Buzz words 8/9/10 5:37 PM
Meissner corpuscles/krause end bulb: in papillary layer of dermis
Langer lines, pacinian corpuscles, ruffini corpuscles: in reticular
layer of dermis
Ceruminous (wax glands), glands of moll, mammary glands: types
of apocrine glands
Acne ass. Glands: sebaceous glands
Coral red fluorescence of scales under wood light: Erythrasma
Corynebacterium (esp minutissimum): erythrasma
Ecthyma: severe form of impetigo
No regional lyphadepathy, little or no redness, often on face: bullous
impetigo
Complication is acute glomerulonephritis: impetigo
Impetigo of brockhart or barbers itch: s.aureus folliculitis
Sty: s aureus folliculitis
Hot tub folliculitis org: pseudomonas
Lymphatic streaking: Superificial bact inf that extends into
lymphatics and primarly in adults: erysipelas
How to differentiate erysipelas from cellulitis: cellulitis margins are
indistinct while erysipelas are sharply demarcated
Verruca vulgaris: common warts: rough surface
Type of wart with smooth surface: flat wart or verucca plana
Wart on eye lid: filiform
Condylomata acuminata: genital or venereal warts
Poxvirus causes this: molluscum contagiosum
Bimodal age distribution: molluscum contagiousum
Henderson-patterson bodies: molluscum contagiosum
Lesion with a central pit: molluscum contagiosum
Lateral borders of feet: moccasin type tinea pedis
Does not involve the scrotum: : tinea cruris
Zoophilic or anthropophilic: tinea corporis
Most common mycotic inf in children: tinea capitis
Endotrix: black dot type of tinea capitus
Mousy odor: favus type of tinea capitis
MCC of diaper rash: cutaneous candidiasis
Enlarged in all three dimension: nodule
Evanescent, edematous and flat elevation: wheal (hives)
Tree bark: lichenification
Pus involving subQ: abscess
Shallow abrasion with no dermal involvment: excoriation
Cicatrix: scar
Eschar: type of ulcer
Over production of keratin: ichthyoses
Mutation in ATA2A2 on chromosome 12: darrier dz (keratosis
follicularis)
Mic app: papule shows cleft above basal layer (suprabasalar cleft) cone of compact keratotic material: darrier dz (keratosis follicularis)
Karatosis follicularis aka: darrier dz
Pink papules with silvery scales: psoriasis
Oncycholysis and nail pitting: psoriasis
Mic app: acanthosis with elongated rete ridges, paraketatosis and
absence of granular cell layer, mitosis above basal layer, collection
of neutrophils (munro abscesses within superficial ep: psoriasis
Munro abscesses: psoriasis
Koebner phenomenon: psoriasis
Suprabasalar blisters with intercellular deposits of IgG and
complement: pemphigus vulgaris (PV)
May be ass with MG or Lupus: pemphigus vulgaris
Flexors and supepithelial bullae: bullous pemphigoid
Linear BMZ of IgG and complement: bullous pemphigoid
Ass with gluten sensitivity: dermatitis herpetiformis
Mic app: neutrophils, eosinophils, and fibrin at tips of dermal
papillae: dermatitis herpetiformis
Extensors and oral no oral mucosa: dermatitis herpetiformis
Flexors and yes oral mucosae: bullous pemphigoid
Granular deposits of IgA at tips of dermal papillae: dermatitis
herpetiformis
Iris lesions: erythema multiforme
Stevens Johnson syndrome: erythema multiforme
Malar area rash: chronic cutaneous (discoid) erythematousus
Butterfly rash: acute SLE
Wickham stiae: lichen planus:
Violaceous papules with white dots or lines: Wickham straie in
lichen planus
Saw tooth pattern of deep surface of ep: lichen planus
Colloid or civatte bodies: lichen planus
Liquefaction degeneration at DE junction: lichen planus
Irritating rash characterized by wheal formation: urticaria
Mic app: scanty perivascular infilitrtates of mononuclear cells, no
increase in mast cells: urticaria
Allergic rxn to eggs: angioedema. Aka giant urticaria *under
urticaria)
AD disorder due to mutation in C1-esterase inhibitor: hereditary
angioneurotic edema *under urticaria)
Affects lower extremities at sites of pressure: cutaneous necrotizing
vasculitis
Spongiosis: allergic contact dermatitis
Silicon breast implant rxn: granulomatous dermatitis
Disorder of dermal CT: scleroderma
Sweat glands and hair follicles are obliterated: scleroderma
F:M 4:1 with non pitting edema of hand or fingers: scleroderma
Mask like expressionless with late stage of thick dense fibrotic and
fixed skin to underlying tissue: scleroderma
No vasculitis and no scaring: erythema nodosum
Most common form of panniculitis: erythema nodusum
Mic app: CT septa widening with edema, fibrin deposition and
neutrophilic infiltration, later replaced by lymphohistiocytic
infiltration with giant cells and eosinophils with (no vasculitis):
ERYTHEMA NODOSUM
Disorder of panniculus with vasculitis and scars: erythema
induratum
Mic app: necrotizing vasculitis affecting small to medium sized
arteries and veins in deep dermis and subcutis followed by
granulomatous inflammation and necrosis: erythema induratum
Degenerative dz of articular cartilage: OA
Oligoarticular: affects few joints: OA
Eburnated: oa
Osteophytes: joint mice: OA
Heberden nodes: OA
Osteophytes in DIP: Heberden nodes: in OA
Pain and guarding of joint: synovial inflammation in OA pain
Pain and bracing of joint: capsular inflammation (capsultitis) in OA
pain
Radiculopathy: osteophytes (bony spurs) in OA pain
Chronic inactivity can lead to muscle weakness and disability: OA
pain
Muscle spasm secondary pain: IN OA
Non suppurative inflammatory and proliferative synovitis: RA
Autoimmune chronic INFLAMMATORY CT dz: RA
Immune complex formation: RA
Prostaglandins, leukotrienes, lysosomal enz, and ROS: products of
neutrophils in RA
Pannus: RA
Hyperplastic synovium rich in inflammatory cells: pannus
Rice bodies: RA: which are organized fibrin
Mass of synovium and synovial stroma consisting of inflammatory
cells, granulation tissue, and fibroblasts: pannus in RA
Systemic amyloidosis: extra-articular involvement in RA
Vaculitis and inflammatory lesions in pericardium, myocardium,
lungs, pleura, peripheral nerves, and eyes: extra-articular
involvement in RA
subQ Rh nodules: extra-articular involvement in RA
hypergammaglobulinemia: RA
Rf is anti what: IgG
Ass with diuretics/aspirin/ and nicotinic acid: secondary gout* less
likely)
Chronic hemolysis, polycythemia, leukemia, and lymphoma:
secondary gout (most commonly)
Tophus: chronic gout
Most imp complication in gout: renal dz : acute uric acid
nephropathy, nephrolithiasis, or chronic urate nephropathy
Big toe: acute gout
Chondrocalcinosis: pseudogout
Calcium pyrophosphate crystals: pseudogout (chondrocalcinosis)
Hydroxyapatite arthropathy: pseudogout (chondrocalcinosis_
Legg calve Perthes dz: avascular necrosis of femoral head in kids
Kohler dz: avascular necrosis of navicular bone
Gaucher dz: avascular necrosis
Steroid administration: avascular necrosis
Drugs:for a while
Metabolism is saturable: aspirin
Irreversible inhibitor of platelets: aspirin
Tx for salicylism: activated charcoal , correct A/B abnormalities/
alkalize urine
Can worsen gout: aspirin
Reys syndrome: aspirin
Dental pain/ oa, or RA: diflunisal (Dolobid)
Tx for Tylenol overdose: gastric lavage with addition of sulfahydryl
cmps to replenish glutathione: N-acetylcystein (mucosil)
Facilitiates closure of PDA: indomethacin (Indocin)
Alternative for PDA closure if indomethacin not available: ibuprofen
(advil, motrin)
FAP and inhibits dev of colon, breast and prostate cancer: Sulindac
(Clinoril)
Gi effects, thrombocytopenia, agranulocytosis, nephrotic syndrome:
Sulindac (Clinoril
This NSAID can close PDA not recommended in preggos: ibuprofen
(motrin/advil)
Increased CV risk: naproxen (aleve/naprosyn)
May antagonize bradykinin: ketoprofen (orudis)
NSAID that also inhibits TNF a and NO SYNTHESIS: flurbiprofen
Dental bone implant preservation: flurbiprofen (ansaid, Ocufen)
Cogwheel ridgitidy, ataxia, tremor, myoclonus: flurbiprofen (ansaid,
Ocufen)
RA + juvenile form. Adverse is nervousness and anxiety: tolmetin
(Tolectin)
Used for post opt pain in place of morphine (opoids): ketorolac
(Toradol)
Long half life and only single dose NSAID: piroxicam (Feldene)
Cox2 selective combo with misoprostol: diclonfenac (Cataflam,
voltarin)
Cox2 sel for long term tx for RA, OA or ankylosing spondylitis:
diclonfenac (Cataflam, voltarin)
Inhibits thymidylate synthetase, and aminoimidazolecarboxamide
ribonucleotide transformylase: methotrexate (Rheumatrex)
Crosslink’s DNA/ metabolized to phsophoramid mustard/ a DMARD:
cyclophosphamide (cytoxam, neosar)
Decreases IL1, IL2: cyclosporine (Sandimmune, neoral)
Avoid drinking grapefruit juice: cyclosporine (Sandimmune: neoral)
More frequently used for transplantation than RA: a type of DMARD:
azathioprine (Imuran)
The monoclonal antibody RA (DMARD) tx: rituximab (Rituxan)
The monoclona antibody RA (TNFa inhibitor): adalimumab (Humira)
Potent tnf alpha inhibitor that inhibits macrophages: infliximab
(Remicade)
Can worsen MS: infliximab (Remicade)
Tnf a inhibitor that can tx ulcerative colitis and juvenile arthritis and
is an recombinant fusion protein: entanercept (Enbrel)
RA tx that prevents secondary costimulatory signal for t cell:
abatacept (Orencia)
Reduces IgA and IgM and suppresses t cell activity: sulfasalzine
(Azulfidine)
Inhibits dehydrooraotate dh and do not combo with methotrexate:
leflunomide (Arava)
RA tx that can cause aplastic anemia after IM injection: gold
formulation
Bact inf of skin: favreau
Honey colored crust: bullous impetigo
Acute nephritis dev in 2-5% and streptococci induced cases acute
nephritis in 10-15%: non bullous impetigo
Well defined vs less defined: impetigo to cellulitis
Tx for impetigo: self limiting or mupirocin (bactoban)
Tx for generalized impetigo: oral atb DOC: cephalexin (Keflex)
Tx for generalized impetigo is penicillin allergy: azithromycin
Ear and lower leg are high areas of infection: cellulitis
Periorbital cellulitis: a medical emergency
Most common pathogens causing cellulitis: GRp A stretococcus,
straph aureus
How to dx cellulitis: clinical dx
Tx for what dz: elevate arm above heart: cellulitis and erysipelas
What to do if you suspect gas gangrene in cellulitis (clostridium
perfringes): xray to check for gas
Lymphatic streaking: think erysipelas
80% of cases of erysipelas caused by : streptococci
FLD for erysipelas: penicillin
Drug allergy to penicillin to tx erysipelas: erythromycin or
azithromycin
This occurs in pts on long term atb tx for acne: Gram – folliculitis
Hot tub folliculitis: pseudomonas
Appears 8-48 hrs after exposure to dirty water or wet suits. :
pseudomonas folliculitis
Seen in young adults/females and eruption is due to follicular inf by
malassezia furfur (lipophilic yeast): Pityrosporum folliculitis
Dx of folliculitis is usually by: hx and PE alone
Most common pathogen for folliculitis: s. aureus
Tx for s aureus folliculitis: oral anti staph atb
Tx for pseudomonas folliculitis: usually self limiting or Cipro
Eosinophilic folliculitis tx: isotretinoin, metronidazole, UV-B
phototherapy, indomethacin, itraconazole
Tx for Pityrosporum folliculitis: topical antifungal cream/shampoo
Most common cause of carbuncles: s. aureus
Thongs: carbuncles
Can you transmit yeast by sex: yes
Satellite lesions: think candidiasis
Yeast inf in penis: candida balanitis
Ddx for tinea vesicolor that is hypopigmented and no scale: vitiligo
Fungal inf of palms and finger webs with scaling and erythema:
tinea manum
Red plaque with a silvery scale: psoriasis
Gender etiology of psoriasis: m=f
Auspitz sign: pinpoint bleeding seen when cutting into edge of
psoriasis
Tx for psoriasis: topical steroids
100% TQ: herald patch: pityriasis rosea
5 ps of lichen planus: pruritic, purple, papules, planar, polygonal
khin skeletal system
cyst that contains amorphrous protein like material and lined by
fibrous tissue, few giant cells, hemosiderin-laden macrophages,
chronic inf cells and reactive bone: solitary(unicameral bone cysts)
curettage and insertion of bone chips-curative: solitary(unicameral
bone cysts)
most tumors appear near: metaphysis
more than 80% of primary tumors occur in what bones: distal femur
and proximal tibia
involves skull and facial bones such as frontal sinus (most common
site): osteoma M>f
gardner syndrom: osteoma M>F
xray finding of central radiolucent area (nidus): osteoid osteoma
M>F
non painful and predominantly affects vertebrae and long bone and
m=f: osteoblastoma (giant osteoid osteoma)
most common primary malignant tumor representing 1/5th of all
bone malignancies: osteosarcoma (osteogenic sarcoma)
may arise in ass with pagets, fibrous dysplasia, enchondroma, or
previous exposure to irradiation: osteosarcoma (osteogenic
sarcoma)
tumor suppressor genes such as p53 or 2/3rd due to Rb mutation:
osteosarcoma (osteogenic sarcoma)
conmans triangle: osteosarcoma (osteogenic sarcoma)
sun ray appearance on xray : osteosarcoma (osteogenic sarcoma)
rare variant of osteosarcoma that occurs more in females:
juxtacortical osteosarcoma
non hereditary disorder with multiple enchondromas in metaphysis
and diaphysis of various bones: ollier dz (enchondromatosis)
familial multiple form ass with hemangiomas in skin and viscera:
Maffucci syndrome
codman tumor: aka: chondroblastoma: M in second decade of life
and affects epiphyseal areas of bones
ass with trisomy 7: chondrosarcoma
ass with rearrangement of chrom 17: high grade chondrosarcoma
large bossilated mass that surrounds the base of an
osteochondroma and invasion in bone: peripheral chondrosarcoma
slow growth may lead to compression of lumbosacral plexus and
thus paraplegia: peripheral chondrosarcoma
gender preference for juxtacortical chondrosarcoma vs
osteosarcoma: chondro: M vs osteo: F
snowstorm appearance: condrosarcoma
most frequent primary sites are Males: bronchus/prostate and F:
breast: metastatic bone tumors
first sign of neoplasm’s: back pain
periosteal onion skinning and sunburnt pattern of maligant
metastatic bone tumor: ewing sarcoma
tumor cells contain abundant glycogen: ewring sarcoma
osteoclastoma:aka: giant cell tumor of bone
soap bubble appearance : osteoclastoma:aka: giant cell tumor of
bone
spindle stromal cell: osteoclastoma: aka giant cell tumor of bone
osteitis deformans: paget dz of bone
irregular thickening and softening of the bones: paget dz: osteitis
deformans.
Slow virus: Paramyxovirus: paget dz: osteitis deformans.
Woven bone with mosaic pattern: initial stage of paget dz: osteitis
deformans.
Coxa vera and anterior bowing: paget dz: osteitis deformans.
Deafness, height distortion, coarsening of facial bones (leontiasis
ossea) paget dz: osteitis deformans.
Serum alkaline phosphatase elevated is the most useful lab test to
dx: paget dz: osteitis deformans.
Ass with skin pigmentation and endocrine distrubances: fibrous
dysplasia
Most common endocrine abnormality: Albright syndrome
Pigmented macules (café au lait spots) usually over buttock, back
and sacrum: Albright syndrome
Histological features of woven bone that looks like Chinese
characters: fibrous dyspasia
Clubbing of digits: hypertrophic osteoarthropathy
Few hem questions:
Pt has some mile petechia and bruising: this means what: low
platelet count
TQ: hallmark of the dz requires both pancytopenia and acellular
bone marrow filled with fat, sustained (often irreversible), and
caused by stem cell injury: aplastic anemia: *the PLASTIC surgeon
put the FAT in the PAN.
What is the genetic predispositon for aplastic anemia: HLA-DR-2 (
exposure to benzene: Aplastic anemia
name the dz: pancytopenia, acellular (or marked hypocellular ) bone
marrow, CFU-GM and BFU-E decreased, decreased long term culture
initiating cells to less than 1%, flow cytometry rules out ALL or hairy
cell leukemia: Aplastic anemia
how would you dx apastic anemia: get a CBC to see pancytopenia,
get a BM biopsy to see fatty infiltration
whats the most imp tx for survival of aplastic anemia: bone marrow
transplantation
whats the prognosis of Aplastic anemia: now 75%, but 25% severe
graft vs host (GVH), 15% relapse, 25% progress to PNH, MDS, or
AML
what are the Ddx of aplastic anemia: (4): (AMP-H): ALL,
myelodysplastic syndrome, PNH, Hairy cell leukemia
what dx test of choice for AA: bone marrow aspirate and biopsy
what are you looking for on the bone marrow aspirate or biopsy:
spicules with empty fatty spaces with hypo-cellularity
whats the best therapy for AA: remove oftending agent, blood
transfusion, BM transplant, immunosuppresents (cyclosporine, anti-
thymocye globulin), corticosteroids, bone marrow stimulants (GCSF
or other cytokinesn, epo. Tincture of time
myelo means: all formed elements of blood
schisto means: fragmented cells
pancyto means: all the cells
pioklio: change in shape
reticulo: early rbc precursors
blastic: early
what does a CBC test: HHRRWWPB: hct, hgb, rbc count, rbc indices,
wbc count, wbc types (differential), platelet count, blood smear
MCV: average RBC size
MCH: Hb amt per RBC
MCHC: Hb amt relative to size of cell (hb concentration) per RBC
ICT vs DCT: ICT= indirect chulmbs test= tells you atc is in the
plasma vs DCT: direct chulmbs test= atb on the RBC: D or D**
4 d’s of quantitative thrombocytopenia: decreased production,
dilution, distribution, increased destruction.
Myeloid vs lymphoid: myeloid is from the bone marrow where as
lymphoid is from the LN, thymus and spleen. Myeloid induces:
precursors for granulocytes (neutrophils, basophils, eosinophils,
monocytes, rbc, platelets. Lymphoid induces LYMPHOCYTES
Acute vs chronic: acute young malignant cells: predominaly blasts
vs chronic in which mature malignant cells predominate
Dz of adults, stem cell disorder, mature cells predominate, and BM
shows “hypercellularity”: myeloproliferative disorders
TQ:Multiple myeloma, light and heavy chain dz, benign monoclonal
gammopathy, walenstroms macroglobulinemia, amyloidosis,
plasmacytoma, plasma cell leukemia, monoclonal gammopathy of
undertermined significance Plasma cell disorders.
Pt (prothrombin time) or PTT (partial thromboplastin time:
o Extrinsic pathway:factors 2, 5, 7, 10 or fibrinogen: PT
o Factors: 8, 9, 11, 12 and rarely von willebrands factor: PTT
TQ: protein C or protein S def or HIT antibody: hypercoagulable
states
Stopped at start of hemolytic anemia lecture
Pentad of TTP: microangiopathic hemolytic anemia,
thrombocytopenia, renal failure, mental status changes, fever
Tx for TTP: plasma exchange
Panavellil heme dugs
Tx for iron toxicity: deferoxamine”
Tx for ACD: EPO
Tx for CRF: chronic renal failure: EPO
Tx for bone marrow deficiency states: CSF (colony stimulating
factor)
Production of RBC by what molecules: EPO
Where is epo produced: kidney
What is epo’s trigger for release: low o2 content which then
stimulates__________ (stem cells to differentiate to proerythrocytes) ,
increases in mitosis, increase release of reticulocytes, induces hb
formation
Where is the majority of iron absorbed: in the duodenum in the
ferrous state (fe2+)
Transport plasma protein that delivers iron the bone marrow for the
incorporation into RBC hb: transferrin
Where is iron stored: in macrophages in the reticuloendothelial
system as ferritin or hemosiderin
What do RBC break down into: aa and porphyrin
Bilirubin is released into the plasma where it binds to albumin and is
transported to the LIVER for what rxn: glucoronide conjugation and
excretion via bile
Tx for IDA: iron deficiency anemia: ferrous sulfate, ferrous fumarate,
ferrous choline citrate, ferrous gluconate, polysaccharide iron
complex
How does orange juice affect IDA tx:2x the absorption
How does tea or coffee affect IDA tx: ½ the absorption
Therapeutic doses of iron increase hb by : 1g/wk
Parental iron forms: iron dextran, INFeD, dexferrum, pri-dextra,
sodium ferric gluconate complex-ferrlecit, iron sucrose (Venofer)
in IDA pts with MALABSORPTION SYNDROMES or pts INTOLERANT
TO ORAL IRON PREps a combo of what is given: ferric hydroxide and
dextran (iron dextran) containing 50mg/ml iron im or iv by multiple
slow injections
what do the reticuloendothelial cells do to the iron dextran:
phagocytize it
what is an adverse affect of IM injection of iron: tissue necrosis or
atrophy
what are pre existing immune conditions that my predispose pts for
high risk adverse rxns for parenteral iron forms: SLE and RA
the newer form of parenteral iron for chronic renal disease: Ferrlecit
(sodium ferric gluconate complex)
exchange mechanism seen in which drug: iron sucrose (Venofer)
what is the form indicated for IDA in chronic hemodialysis pts who
are receiving EPO therapy: iron sucrose (Venofer)
whats an advantage of parenteral vs oral: faster
GI upsets, discoloration of feces (black): adverse rxns to parenteral
iron
What are the mech of toxicity for iron toxicity: 1 direct corrosive
effec on mucosal tissue resulting in necrosis and perforation, 2.
Cellular toxicities due to lactic acidosis and necrosis
Clinical presentation is bloody diarrhea, massive fluid loss and blood
loss into gi tract causing shock, renal failure and death: iron toxicity
What is metabolic acidosis ass with: iron toxicity even after survival
What is the chelating agent used for: its deferoxamine and its used
for iron toxicity
Orange or pink red urine: tx for iron toxicity: deferoxamine
Cyanocobalamin: b12
Cobalamin is absorbed by both: intrinsic factor dep and
independent routes
Malfunction of the parietal cell: decreases intrinsic factor which
decreases cobalamin absorption (b12) which causes pernicious
anemia
Pernicious anemia: b12
Acquired vs congential pernicious anemia: acquired if decreased
production of intrinsic factor. Congenital if dysfunction of intrinsic
factor
B12 transport protein: transcobalamin II
Addisonian anemia aka: pernicious anemia
Tx of pernicious anemia (addisonian anemia): oral b12 therapy
Which anemia can be tx with intranasal formulations: vit b12 def
with intranasal cyanocobalamin formulations.
Whats hydroxocobalamin: preferred for b12 anemia due to highly
protein bound and remains longer in circulation
Hyperuricemia, hypokalemia, and sodium retention and rebound
trambocytosis leading to thrombotic events seen in: b12 induced
reticulocytosis
Schilling test: test to determine b12 def
Whats ass with colon cancer: excess homocysteine seen in folic acid
def anemia
Two drugs that can block dihydrofolate reductase and lead to folic
acid def anemia: methotrexate and trimethoprim
Are folates toxic: no known toxitcity
What is the major difference seen in folate and b12 def: in folate
def there is an absence of neurological manifestations
Tx for megaloblastic anemia: folate and cyanocobalamin
What is the glycosylated protein: EPO
Tx of renal failure anemia, aids anemia, ACD such as metastatic
cancer and RA: epo
How is epo administered: IV or SUBQ
Epo effect of rbc: increases levels
Epo effect on hct: increases and activates syn of hb
What is epo not indicated for: pts needing immediate correction of
severe anemia
What is the major side effect of epoetin therapy: elevated diastolic
pressure (Hypertension)
What causes seizures, thrombosis or iron deficieny: EPO
What does Neupogen (for wbc def) do: increases total neutrophil
count
Tx for white blood cell def: G-CSF (Neupogen), Leukine, or neulasta)
N. ditchek rheumatoloical problems lecture
What should lead to correct dx in 85% of rheumatologic problems:
hx and pe.
Functia laesa: loss of function
Rigor: stiffness
Erythema, soft tissue swelling, sausage digits, and nail changes
seen in : psoriatic synovitis
Podagra: gout in the big toe
Vesicles and pustules due to gonococcal arthritis: pustular synovitis
Pip vs dip: pip: bouchards, dip : heberdens: both in OA
Non inflammatory: OA
Inflammatory : RA, SLE, psoriatic arthritis
Features of arthritis: inflam, pain in all motions (active and passive),
pain around joint, and decreased rom
What are some routine arthritis screening tests: acute phase
reactant (ESR and C-RP), RF, and ANA
ANA
PATTERNSxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxx
o Hemogenous histones/dna: SLE or drug effect
o Speckled ENA: MCTD, SLE, sjogrens syndrome,
poly/dermatomyositis
o Nucleolar RNA ass angtigen: scleroderma
o Peripheral (rim) DNA nuclear antigens: SLE
o Centromeric nuclear antigens Limited scleroderma (crest
syndrome)
AUTOANTIBODIES IN RHEUMATIC DZ
Anti-dsDNA: SLE
Anti-histone: SLE, drug induced lupus
Anti-ENA: (sSm or RNP (ribonucleoprotein): SLE MCTD or SLE
Anti ss-a/ro: SLE, neonatal lupus, sjorgrens syndrome
Anit ss-b/ro: sjorgrens, sle, neonatal lupus
Anti centromeric: limitied scleroderma (crest syndrome)
Anti scl-70: diffuse scleroderma
Anti jo 1: poly/dermatomyoctis, raynauds phenomenon’s
Anti PM scl: polymyositis/ scleroderma overlap syndrome
Anti mi 2: dermatomyositis
Special arthritis tests:
o Complement levels: CH 50, C3, C4
o HLA: b27, D locus alleles
o Antineutrophil cytomplasmic antibody (ANCA)
o Cryoglobulins
o Lyme titers
Routine lab studies for rhematologic studies
o Cbc
o Chemistries
Glucose
Liver function test
Cpk
Uric acid
Urinalysis
What are the causes of hemarthrosis (bleeding into joint)
o Trauma
o Bleeding disorder
o Villonodular synovitis
o Pyrophosphate arthropathy
o Charcot joint
o Resolving inf
o Contamination during aspiration
Synovial effusions
o Clear colorless viscous with leukocytes <200, <25% pmn:
normal
o Clear, yellow, viscous with leukocytes 200-2000, <25% pmn:
non inflammatory: ie OA
o Cloudy, yellow, watery , glucose may be low with leukocytes
2000-100,000, >50% pmn: inflammatory: RA
o Purulent, glucose very low with leukocytes >80,000 and
>75% pmn: septic: bact inf/fungal inf, mycobacterial inf, or
acute crystal synovitis
Types of crystals found in acute gout and topheacous gout
monosodium urate monohydrate
Calcium pyrophosphate in : pseudogout, destructive arthropathy
Basic calcium phophate: acute calcific periarthritis, acute arthritis,
destructive arthropathy (Milwaukee shoulder/knee)
Calcium oxalate: acute, subacute arthritis
Lipids (fat): acute arthritis
Cholesterol: asymptomatic
Negative birefringence: urate crystals in gout
Positive birefringence : calcium pyrophosphate crystals in
pseudogout
Vasculitis
Whats the key to dx for vasculitis: clinical suspicion
Pulseless, blindless, cva, and syncope: large vessel symptoms
CAD, Renal artery CVA, mononeuritis (writs/foot drop): medium
vessel
Palpable purpura and raynauds/ gangrene: small
The only medium vessel one: polyarteritis nodosa
The two large vessel ones: takaysu (rare) and most common
temporal arteritis
Past TQ: causes of leukocytoclastic vasculitis aka hypersensitive
vascultis or allergic vasculitis: viral/ bact/ atypical, meds, lupus,
idiopathic, insect bites
Livedo reticularis: mottled erythema seen in : small vessel vasculitis
Necrotizing (caseating) granulomatous vasculitis, 1.3:1 in M;f, >95%
in caucasians in 20-40s with tx of glucocorticoids +
cyclophosphamide: wegeners granuloamatosis (sm vessel)
What is ELK: main sites for wegeners granulomatosis: Ent/ upper rt:
optic, otic, sinusitis, Lower rt: lungs, Kindey
Whats the lab test for wegeners granulomatosis: C-ANCA
TQ: what is Henoch Schonlein tetrad: (Ga^2p)
o Glomerular nephritis
o Ab pain
o Arthritis
o Purpura
Markers of inflammation: Elevated ESR and C-RP
Hematologic changes in vasculitis: leukocytosis usually with
neutrophila, anemia, thrombocytosis
High RF with low complements: hepatitis related PAN, RA, or
cryoglobulinemic vasculitis
Past TQ: ANCA ass dz: wegners, microscopic polyangiitis, chrug-
strauss syndrome, drug induced anca ass vasculitis
Weight loss >4kg, livedo reticularis, testicular pain or tendernous,
myalgias, weakeness or leg tendernous, mononeuopathy or
polyneuropathy, diastolic bp>90, elevated BUN/creatinine, hep b
virus, arteriographic abnormality, bx of small or medium artery
containing PAN: polyarteritis nodosa (the only medium vessel
vasculitis)
Pts at least 50 and usually white, pain at least 1 month in shoulders
and pelvis, severe morning stiffness and gelling: polymyalgia
rheumatica (large vessel)
HA, diplopia, anaurosis, jaw claudication: cranial arteritis
Giant cell arteritis aka: temporal arteritis
TQ:Elevated Westergren esr >59: temporal arteritis
Tx for temporal arterits: high dose steroids
Dx of temporal arteritis: must biopsy temporal arteries bilaterally
Fragmentation of the intima layer of vessel: temporal artertis
TQ: the most dangerous outcome of TA: ischemic optic neuritis:
demands immediate tx with high dose steroids
Jones skeletal MSK pathology
Connective tissue covering of bone: periosteum
Mononuclear cells that syn collagen and ground substance:
osteoblasts
Osteoblasts that become embedded and incorp into bone matrix
osteocytes
Multinucleated cells found along cortical endosteal surface and
trabeculae in scalloped bays called howships lacunae osteoclasts
Howships lacunae: osteoclasts locations
Haversian system: compact bone
Basic unit is trabecula: cancellous bone
Collegen laid down in parallel array: lamellar bone
Collagen laids down haphazardly : woven bone
Endochondral ossification: long bones
Intramembranous ossification: flat bones
Dz characterized by increased porosity of skel reduced bone
mass: osteoporosis
Bone mass determined by : vit D receptor allele, genes for collagen
1A1, estrogen rec, Insulin like growth factor 1 and its binding
protein, physical activity, diet, hormones
When is maximal skeletal mass attained: after 3rd or 4th decade
When does postmenopausal osteoporosis occur: 1 decade after
menopause
Decreased estrogen levels in post menopausal osteoporsis results
in: increased secretion of IL1, IL6 and TNF by blood monocytes and
bone marrow cells (these cytokines activate osteoclasts)
What causes senile osteoporosis: osteoblasts reduced replicative
and biosynthetic potential
Morphology: affects the cancellous compartment of vertebral
bodies: post menopausal osteoporosis
Trabeculae are thinned: post menopausal osteoporoiss
Cortex is thinned: senile osteoporois
Haversian systems are widened: senile osteoporosis
Cannot be detected in plain radiographs until 30-40% bone mass is
lost: osteoporosis
Best dx of osteoporosis: dual energy absorptiometry and
quantitative computed tomography which measure BONE DENSITY
Major function is maintenance in normal plasma levels of ca and
phorphorus: vit D
This is required for absoption of ca from small int: vit D
Vit d def results from: dietary deprivation, fat malabsorption, lack of
sun exposure, chronic renal failure:
Vit d def or phosphate depletion in kids: rickets
Vit d def or phosphate depletion in adults: osteomalacia
Deranged bone: rickets
Undermineralized bone: osteomalacia
Wide osteiod seams: rickets or osteomalacia
Rachitic rosary (swollen costochondral junctions of ribs): rickets
Pigeon chest: rickets
Bowing of legs and arms: rickets
Xray will show thickend, irregular, lobulated epiphyseal plate:
rickets
Osteopenic x ray finding: osteomalacia
Compression fx and decreased bone thickness: osteomalacia
Dev of secondary hyperparathyroidism: osteomalacia (due to low
serum ca which stimulates parathyroid glands)
What causes 80% of primary hyperparathyroidism: adenoma of the
parathyroid gland
Osteitis fibrosa cystica: refers to the anatomical changes of severe
hyperparathyroidism
Morpholgy is increased osteoclast affects cortical bone, dissecting
osteitis (in cancellulous bone what looks like railroad tracks), xray
finding of osteopenia (decreased bone density), marrow space
replaced by fibrovascular tissue, and widening of haversian canals:
hyperparathyroidism
Bone loss predisposes to microfx and secondary hemorrhages:
hyperparathyroidism’s
Brown tumors: hyperparathyroidism
Hallmark is generalized osteoitis fibrosa cystica: von
Recklinghausen dz of bone which includes increased bone
cell, peritrabecular fibrosis, and cystic brown tumors: severe
hyperparathyroidism
CRF results in phosphate retention and hyperphosphatemia: renal
osteodystrophy
What does the hyperphosphatemeia in renal osteodystrophy trigger:
secondary hyperparathyroidism hypocalcemia develops
intestinal ca absorption decreased due to high levels of phosphorus
inhibiting renal hydroxylase, PTH secretion increases with a
decrease degration and excretion of PTH which results in secondary
hyperparathyroidism which increases osteoblast activity. Metabolic
acidosis is ass with renal failure which stimulates bone resorption
and the release of calcium hyroxyapatite from matrix
Aluminum: renal osteodystrophy
What are sources of aluminum: dialysis sol. Or oral al phosphate
binders.
What does aluminum do: interferes with deposition of calcium
hydroxyapatite and thus promotes osteomalacia
Kimmel lecture 1 RA and OA
Genetic predisposition: OA
Worse with use. Improves with rest: OA
Bony changes localized to weight bearing surfaces/ joints: oa
Osteophytes: oa
Hylans: tx for oa
Decrease life expectancy: steroids
Increase life expectancy: DMARDS
7 criteria for RA: (must be present for >6 wks
o 3 jnts
o hands and wrists
o AM stiffness
o Symmetry
o Nodules
o Rf +
o X ray changes
Immunological disorder: RA
Worse at rest better with use, inflammatory/ systemic complaints:
RA
Lab findings for RA: RF, CCP, ESR, CRP
Xray finding for RA: juxta-articular osteoporosis, jt space narrowing
diffusely and errosions
DMARDS: increase life expectancy
o Mild:
plaqenil (hydroxychloroquine)
azulfidine (sulfasalazine)
o stronger
Rheumatrex/Trexall (methotrexate)
Avara (leflunomide)
Biological DMARDS
o Inhibitors of TNF
Soluble receptor: etanercept (Enbrel)
Monoclonal ab: adalimumab (Humira)
Golibumab (simponi)
cerulizumab (cimzia)
infliximab (Remicade)
o IL1 inhibitor: anakinra (kenneret)
o IL6 inhibitor: tocilizumab (actimra)
o B cell inhibtor: rituximab (Rituxan)
o T cell inhibitor: abatacept (Orencia)
o oral biological DMARDS: protein kinase inhibitors
abnormalilty in collagen type II gene (aberrant COL2A1 gene): OA
no ass with HLA: OA
HLA-DR4: RA in white people
HLA-DR1, DR10, DRw6: RA in non white
Life expectancy of RA: 10 years less than normal
In OA which cause decrease in matrix syn: IGF 1, TGF beta
In oh which cause increase in matrix degradation: cytokines, enz,
NO
Major cell type in RA in synovial fluid: neutophils
Major cell type in pannus: t lymphocytes and macrophages
Minor cell types in pannus: fibroblasts, plasma cells, endothelium,
dendritic cells
Crepitus: oa
Polyarticular: RA
Joints involved in primary OA: cervical/lumbar vertebrae, hip,
dip/pip, 1st CMC, knee, 1st MTP
Diagnostic joints for early ra: wrist, 2 or 3rd MCP, MTP
Lab result of ESR <20 : OA
Most specific xray finding for Oa: osteophytes
Can Rf be negative in early RA: yes
When does symmetry of joint involvement occur in RA: late ra
RF+ but ANA -: RA with no SLE
Spondyloarthropathy lab finding: RF- and ANA –
Erosions: RA
Glucosamines use: OA ONLY>>>>>
Fentanyl patches, codeine, oxycodone, propoxyphene, or morphine:
opioids
Which opioid is long acting an will have fewer CNS side effects and
less addictive potential: morphine
Cox-2 specific: celecoxib (celebrex), vioxx and Bextra
Selective cox 2: meloxicam (Mobic), and nabumetone (Relafen)
Hylans: hyalgen, Synvisc, orthovisc, Supartz, euflexxa
What are the indications for hylans: tx of knee or hip Oa in pt unable
to respond to steroid injections. DO NOT USE FOR small joints or
inflam arthritis (RA)
Hypopigmentation: steroid side effect
Diabetes flare: steroids side effect
What are the indications for joint replacement: persitent pain
despite medical PT, loss of joint function, fracture
Absolute contraindications for joint replacement: infection
Relative contraindications for joint replacement: age, obesity, poor
health, poor bone stock, deformities, prior infection
Which ones slow dz progression in RA tx: DMARDS
DMARD side effects TQ
o Macular damage: hydroxycholorquine
o Myelosuppresion, GI: Azulfidine
o Gold oral: myelosuppresion, rash, proteinuria, GI
o Leuflunomide: hepatotoxic, GI no in preggo
o Methotrexate: hepatoxic, pulmonary, myelosuppression, No in
preggo
Contraindications are MS or TB inf, CHF, vaccines (zoster): tna
antagonist
Criteria for dx of adults stills dz:
o All required:
Fever >39 c
Arthralgia or arthritis
Rh factor <1:80
ANA <1:100
o In addition to any of the following
Wbc >15000
Stills rash : faint salmon colored rash on trunk or UE
Pleuritis
Pericarditis
Hepatomegaly or splenomegaly or generalized
lymphadenopathy
Complication of adult stills: hepatic necrosis
Tx for adult stills: biological tx IL1 inhibitor: anakinra (kinneret)
Dx of feltys syndrome
o Seropositive rheumatoid arthritis
o Splenomegaly
o Granulocytopenia
How long should you use contraception after on MTX: 3-6 months
How long should you use contraception after on leflunomide (Arava)
2 years
Khin msk skeletal system
80% of primary bone tumors occur in: distal femur or proximal tibia
frontal sinus: osteoma
gardners syndrome: osteoma
nidus: osteoid osteoma: usually LE and small bones of foot
pagets, fibrous dysplasia, enchondroma or exposure to irradiation
association: osteosarcoma
codmans triangle: subperiosteal elevation seenin osteosarcoma
sun ray appearance on xray: osteosarcoma
codmans tumor: aka chondroblastoma
codmans tumor (chondromblastoma affects
_____________(epiphyseal area) where as chondromyxoid fibroma
affects ___________(metaphyses)
ass with trisomy 7: chondrosarcoma
ass with rearrangement of chromosome 17 high grade
chondrosarcoma
large bossilated mass: peripheral condrosarcoma
gender preference for juxtacortical chondrosarcoma vs juxtacortical
otsteosarcoma: chondro: men, osteo: women
snowstorm appearance condrosarcoma
MC sites for metastatic tumors of bone in M: bronchus and prostate,
F: breast
Onion skinning and sunburnt pattern: ewing sarcoma (a type of
metastatic bone tumor)
Ass with reciprocal translocation t(11;22): ewing sarcoma (a type of
metastatic bone tumor)
Tumor cells with abundant glycogen: ewing sarcoma (a type of
metastatic bone tumor)
Soap bubble appearance: giant cell tumor of bone (osteoclastoma)
Gender pref for giant cell tumor of bone (osteoclastoma): females
slightly
Mic app of giant cell tumor of bone (osteoclastoma): composed of
plump spindle cells among scattered multinuclearted giant cells.
The neoplastic cell is the spindle stromal cell
Irregular thickening and softening of bone: paget dz (osteitis
deformans)
Woven bone with mosaic pattern: paget dz (osteitis deformans)
Vertebral compression, height distortion, coarsening of facial bones
(leontiasis ossea): paget dz (osteitis deformans)
Serum alkaline phosphatase elevated and is the most use lab test to
dx this dz: paget dz (osteitis deformans)
Ass with skin pigmentation and endocrine disturbance: fibrous
dysplasia
Most common endocrine abnormality: Albright syndrome
Café au lait spots over buttocks, back and sacrum: Albright
syndrome
Lucent ground glass appearance and bones appear as Chinese
characters : albrights syndrome
Cellular fibroblastic tissue with scattered lipid laden macrophages
and multi nucleated giant cells, no bone formation or anaplasia:
nonossifying fibroma (fibrous cortical defect
Clubbing of digits: hypertrophic osteoarthropathy
Jones infection inflammatory and reactive arthritic conditions
Most common organisms of infectious arthritis (PEGS3)
o Pseudomonas
o Ecoli
o Gonoccus
o Staph
o Strep
o Salmonella
Main causative agent for infectious arthritis in children and adults:
s. aureas
Same as above but in late adolescence and young adulthood:
gonococcus
If sickle cell more prone to infect arthritis by this organism:
salmonella
Sudden acutely painful, hot and swollen joint, restricted rom, fever,
leukocytosis and elevated ESR: infectious arthritis
Disseminated gonococcal inf sites vs intravenous drug abuse sites:
gon: single joint where as IV drug is axial articulations such as
spine, sacroiliac or sternoclavicular
Chronic progressive/ monoarticular dz occurring in all age groups:
tuberculous arthritis
What joints are affected in tuberculous arthritis: weight bearing
joints
Mycobacterium stain: red (fite stain)
Clinical lab aids in dx
o Gram stain:
Always performed as routine
75% sen for staph
50% sen for most gram –
<25% for gonococcal
o blood cultures
obtained before atb given
positive 50% for staph aureus
less frequently + for other org
o synovial fluid
75-95% sen for non gonococcal joint inf
can be turbid, serosanguineous or purulent
high number of neutrophils
protein/ LDH/ elevated
glucose low
fluid should be examined for crystals to r/o other causes
cultures are + > 90% of time
non inflammatory:
o oa, traumatic arthritis, PVNS
inflammatory
o RA, sle, reiter syndrome, rheumatic fever, acute crystal
induced arthritis
Purulent (infectious) effusion
o Bac/fungal/tb joints
Hemorrhagic effusions
o Traumatic, PVNS, synovial hemangiomas, hematologic
disorders, thrombocytopenia, anticoagulant therapy
Bamboo spin in : ankylosing spondyloarthritis
What are the seronegative spondyloarthropathies (3)
o Ankylosing spondylitis
o Reactive arthritis (reiters syndrome and enteritis ass arthritis)
o Psoriatic arthritis and arthritis ass with IBD (ulcerative colitis
or crohn dz)
What do the seronegative spondyloarthropathies have in common:
ass with HLA-b27
Rheumatoid spondylitis and marie stumpell dz aka: ankylosing
spondyloarthritis
Which one especially affects the sacroiliac joint: ankylosing
spondyloarthritis
Gender preference for spondyloarhtris: men 2-3x>F
What is the SI joint infiltrated with in ankylyosing spondyloarhtritis:
CD4 and CD8 t cells, macrophages and high tnf Alpha
Inflammation of tendinoligamentous insertion sites and ossification
aka syndesmophytes: ankylosing spondyloarthritis
Complications in this dz may include fx of spine, uveitis, aortitis,
and amyloidosis: ankylosing spondyloarthritis
Defined as an episode of non infectious arthritis of the appendicular
skeleton that occurs within one months of a primary inf localized
elsewhere in the body: reactive arthritis
Ass with genitourinary (chlamydia) and GI tract inf (shigella,
salmonella, Yersinia, campylobacter): reactive arthritis
The triad of arthritis, non gonococcal urethritis or cervicitis, and
conjunctivitis is called: Reiter syndrome
Define reiter syndrome: The triad of arthritis, non gonococcal
urethritis or cervicitis, and conjunctivitis
What are most affected joints in reactive arthritis: ankles, knees and
feet often asymmetrical pattern
Sausage like finger or toe: reactive arthritis
Inflammatory balanitis (circinate), conjunctivitis, cardiac conduction
abnormalities, aortic regurgitation: extraarticular involvement in
reactive arthritis
Ganglions, synovial cysts, and osteochondral loose bodies aka:
reactive tumor like lesions
What do the above result from: trauma or denerative processes and
they are more common than neoplasm’s
Involves the synovium of a joint, involves one or more joints
DIFFUSELY, and happens in 20-40s in m=f: pigmented villonodular
synovitis (PVNS)
Occurs as DISCRETE nodule on tendon sheath in 20-40s and m=f:
giant cell tumor of tendon sheath( GCT)
Giant cell tumor of tendon sheath aka localized nodular
tenosynovitis
Grossly these lesions are red brown to mottled orange-yellow: PVNS
and GCT
Resembles a small walnut: GCT
Tangled mat of red brown folds and finger like projections and
nodules in the knee: PVNS
the tumor cells are POLYHEDRAL, moderately sized and resemble
synoviocytes: GCT and PVNS
Hemosiderin depostits, FOAMY macrophaes, multinucleated giant
cells, and zones of sclerosis: GCT and PVNS
pts complains of pain, locking and recurrent swelling of knee: PVNS
Which one is aggressive and painfull and which one is solitary,slow
growing and painless mass: aggressive +painful =pvns. Solitary
slow growing: GCT
Frequently involves the tendon sheaths along wrists and fingers and
is the most common mesenchymal neoplasm of the hand: GCT
Where do synovial sarcomas occur: majority dev in deep soft tissue
in vicinity of the large joints of the extremities, 60-70% occurring in
LE, esp knee and thigh
Biphasic and monophasic: synovial sarcoma
Hallmark is eh dual line of differentiation of the tumor cells (ie
epithelial like and spindle cells): synovial sarcoma
Epithelial like cells are cuboidal to columnar and form glands or gro
in solid cords are aggregates: synovial sarcoma
Spindle cells; synovial sarcoma
Most synovial sarcomas are: monophasic
Immunohistochemistry for synovial sarcoma: + for keratin and
epithelial membrane antigen (EMA)
+ for keratin and epithelial membrane antigen (EMA) : synovial
sarcoma.
Old test tq info:
the can inhibit methotrexate activey and is used as a rescue agent
in mtx toxicity: luecorvin
what is a concern about mtx: concurrent use of NSAIDS deceases
the clearance of MTX so toxicity can dev rapidly: (death from severe
bone marrow suppression) has been linked to ibuprofen/mtx use.
(ibuprofen same as motrin and advil)
causes undersecretion of uric acid:
o dehydration,
o ketosis,
o renal abnor.
o Drug: directics, low dose ASA,
o toxins such as ethanol, or lead,
o hypothyroidism
causes overproduction of uric acid
o ethanol
o def of HGPRT or G6PD
o superactive PRPP synthetase
o Myeloproliferative disorders
o Psoriasis
Clubbing of digits: usually occurs in pts with bronchial carcinomas
but sometimes with pleural tumors, pulmonary metastes,
mediastinal Hodgkin dz, chronic lung inf, and chronic liver dz
Complication of tnf alpha inhibitor injections: activation of recurrent
TB inf
Albrights ass with: polyostotic bone lesions, endocrine dysfunctions,
acromegaly, cushion syndrome, hyperparathyroidism, pigmented
macules (café au lait spots) over butt, back and sacrum, Chinese
characters
What does colchicine tx: attack of gout
Sunburst or sun ray vs sunburnt:
o sunburst/ray: osteosarcoma (osteogenic sarcoma)
o sunburnt pattern: ewing sarcoma (which is a metastatic tumor
of bone)
histological pattern hallmark in mosaic pattern: pagets dz
best test to dx osteoporosis: DEXA scan
osteopenia defined as BMD score of: btw -1.0 SD and -2.5 SD below
normal
osteoporosis defined as BMD score of : less than -2.5 SD below
normal
benign tumor of young persons, M>F and usually in LE including
small bones of foot. Pain with more sever at night. Xray shows
central radiolucent area (NIDUS) surrounded by reactive sclerotic
bone: osteoid osteoma
crystals found in synovial fluid
o monosodium urate monoydrate: acute gout, tophaceous gout
o calcium pyrophosphate dihydrate: acute psueogout,
destructive arthropathy
o basic calcium phosphate: acute, subacute arthritis
o lipid: acute arthritis
o cholesterole: asymptomatic
anca ass with
o wegeners granulomatous, microscopic polyangiitis, churg
strauss syndrome, drug induces vasculitis
KIMMEL: best way to differentiate btw RA and OA; Hx and PE
Bizarre pleomorphic cells with hyperchromatic irregular nuclei and
abundant mitoses: osteosarcoma (osteogenic sarcoma)
Formation of osteoid and bone in this highly malignant tumor:
soteosarcoma (osteogenic sarcoma)
Inhibits IL1 AND IL2 and don’t drink grapefruit juice: cyclosporine
(Sandimmune, neoral)
Pulmonary hypertension and centromeric ab: limited cutaneous
sclerocerma (LcSSc)
SCL-70: diffuse cutaneous scleroderma (DcSSc)
This scleroderma has a good prognosis: localized scleroderma
Boutonniere deformity : seen in RA with PIP hyperflexed and DIP
hyperextended
Whats a major contraindication for infliximab (Remicade): MS
Wegeners granulomatous constists of
o Nasal or oral inf (oral ulcers or bloody nasal drainage)
o Abnormal chest radiograph (nodules, fixed infiltrates, cavities
o Urinary sediment >5rbc/hpf or RBC casts
o Granulomatous inf on bx (in wall of artery or arteiole,
perivascular or extravascular)
Churg strauss syndrome (allergic granulomatosis and angitits)
o Asthma
o Eosinophilia (>10% wbc
o Mononeuropathy or polyneuropathy
o Transitory pulmonary infiltrates
o Paranasal sinus abnormalities
o Bx with extravascular eosinophils
Polymyalgia rheumatica
o Pt over 50 and usually white
o Muscle pain for at least 1 month in shoulders and pelvic girdle
o Severe morning stiffness and gelling
TA or giant cell arteritis
o Over 50
o New HA
o Temporal artery abnormality (tender or decreased pulse)
o ELEVATED ESR> OR = 50MM/HR
o Abnormal art bx with mononuclear cell infiltrate,
granulomatous inf usually with multinucleated giant cells.
Polyarteritis nodosa
o Weight loss >4kg
o Livedo retularis
o Testicular pain or tendernous
o Myalgias, weakness, or leg tendernous
o Mono or polyneuropathy
o Diasoltic >90
o Elevated bun or creatinine
o Heb B virus
o Arteriographic abnormatily
o Bx of small or medium artery containing PAN
In osteoporosis there is a loss of height and multilevel fx that can
lead to lumbar lordosis or kyphoscoliosis. What would you also find
to be elevated: IL1, IL6 and TNFalpha, rank, and rankL, osteoclast
activity.
o You would find decreased: serum estrogen, response of
osteoprogenitor cells, osteoblast act, and Physical activity
You might find secondary hyperparathyroidism in this: osteomalacia
due to low vit D. (undermineralized bone)
Dry eyes and dry mouth: sjogrens syndrome
Anti-SSA (ro) and anti SSb (la): sjogrens syndrome
Rose Bengal staining/ schrimer test: sjogrens syndrome
Lip bx: sjogrens syndrome
Tx for eyes in sjogrens syndrome: restasis
Tx for oral sx: Evoxac/ Salagen
What should be started within 6 months of dx of RA: dmards
How long to wait for preggo in lefunamide (Arava): 2 years
How long to wait for mtx: 3-6 months
Xray of knee showing medial joint space narrowing: OA
Tx for PVNS or GCT: surgery
Xray finding of thickened irregular and lobulated epiphyseal plate:
rickets (children vit d def)
Rachitic rosary (swollen constocondral junctions of ribs) and pigeon
chest: rickets
Bowing of arms and legs: rickets
Osteopenic x ray pattern: osteomalacia
Compression fx and decrease in bone thickness: osteomalacia
Osteomalacia will also result in: secondary hyperparathyroidism
Eburnation: oa
In gout what is the tophus composed of: macrophages,
lymphocytes, fibroblasts, and foreign body giant cells
Drug that inhibits lymphotoxin alpha: entanercept (Enbrel)
o Also inhibits tnf alpha and beta
Can tx ra, juvenile arthritis, and ulcerative colitis
Adverse: opportunistic inf and lymphomas
Jones tq 100%
o Non inflammatory
Leukocyte ct <3000/uL, few neutrophils
Oa, traumatic arthritis, PVNS
o Inflammatory
Leukocytes ct btw 3000-75,000/uL, 50% > neutrophils
RA, sle, reiter syndrome, rheumatic fever, acute
cystic induced arthritis
o Purulent
Leukocyte ct >50,000, >90% neutrophils
Bac, fungal, tuberculous joints
o Hemorrhagic
Traumatic, PVNS, synovial hemangiomas, hematological
disorders, thrombocytopenia, anticoagulant therapy
Inhibits uric acid formatin by inhibiting xanthine oxidase for tx of
gout: allopurinol
Non purine xanthine oxidase inhibitor for gout: febuxostat
Breaks down uric acid in gout: uricase
To tx attack in gout: colchicine, nsaids, steroids
What are the uricosuric drugs for gout: Probenecid/ sulfinpyrazone
Osteitis deformans: pagets dz
Brown tumors: osteitis fibrosa cystica
Osteoclastoma: spindle shaped cells (aka giant cell tumor of bone)
Sickle cell: salmonella
Late adolescence and young adulthood: s. aureus
Older children and adults: s. aureas
Methotrexate can cause cirrosis
Anti-jo-1: polymyositis/ dermatomyositis
Anti ds dna: sle
Anti-ribonuclear antibodies: (anti-RNP)::: mixed connective tissue dz
(MCTD)
Shaft of a long bone: diaphysis
Where does the ewing sarcoma originate in the bone: medullary
cavity
Which form of scleroderma is ass with pulmonary hypertension:
limited cutaneous scleroderma
Anti-phospholipid antibody syndrome: cardiolipin abs: ass with
abortions
Round hyperchromatic cells with little cytoplasm and virtually no
stroma: ewing sarcoma
Defect in intercellular matrix: mutation in gene ATP2A2 on chrome
12: darrier dz aka keratosis follicularis
Onycholysis: psoriasis
Acanthosis with elongated rete ridges: psoriasis
What are munroe abscesses: neutrophils in psoriaais
What atb is involved in pemphigus vulgaris: IgG
Which skin disorder is ass with mg and lupus: pemphigus vulgaris
Linear BMZ deposits of IgG and complement: bullous pemphigoid
trif
what are the dual roles of articular cartilage hyaline type at ends of
bone:
o friction free movements
o absorption of shock and weight
layers of the synovium
o macrophages with lysosomal enz and dense bodies
o cells secrete hyaluronic acid
the synovial fluid controls:
o diffusion
o ingestion
o secretion of hyaluronate, immunoglobulins, and lysosomal enz
o lubrication by secretion of glycoproteins
what is the main source of nutrients for articular cartilage since it
lacks blood supply: synovial fluid
elastic and high tensile strength: hyaline cartilage
what is hyaline cartilage made up off
o type 2 collage fibers: tensile strength and transmits vertical
load
o water and proteoglycans for: turgor and elasticity and limit
friction
o chondrocytes: syn matrix and digest matrix
layers of chondrocytes from superficial to deep
o tangential or gliding:
elongated, flattened and parrell
o transitional:
chondrocytes are larger, ovoid and more randomly
distributed
o radial zone:
small and arranged in short columns like in the
epiphyseal plate
o calcified zone: Deepest
small chondrocytes and heavily calcified matrix
what controls matrix turnover: chondrocytes
cytokines/growth factors needed for osteoclast differentiation
o IL1, IL3, IL6, IL11, TNF, GM-CSF, M-CSF
Stimulate osteoclast progenitor cells
Participate in Paracrine system in which osteoblast and
marrow stromal cells play central role
Mediators of the paracrine system
o Rank
o rankL
o osteoprotegerin
what types of cells in rank mostly on: macrophage/monocytic
lineage such as pre-osteoclasts
what types of cells is rankL on: cell membranes of osteoblasts and
marrow stromal cells
what initiates osteoclastogenesis: rank binding to rankL
what inhibits osteoclastogenesis: OPG by acting as a decoy that
binds RankL.
Ehler-danlos, marfans or osteogenesis inperfecta: secondary Oa
Achondroplasia, congenital hip dysplasia: secondary Oa
Macro trauma or repeated microtrauma: secondary Oa
Fibrillation (flaking: oa
Subchondral bone cysts: oa
Joint mice: oa
Pain and guarding of the joint: synovial inflammation in oa
Dull aching pain in oa: when full thickness cracks or erosions in
articular cartilage allow joint fluid to be forced through subchondral
bone plate
Pain and bracing of the joint: capsular inflammation
Non suppurative inflammatory and proliferative synovitis: RA
What forms the Rf factor: B cells
What activates the macrophages to produce cytokines in RA: t cells
What activates neutrophils: IC and complement
What are the mediators of inflam and destruction that are produced
by the neutrophils: prastaglandins, leukotrienes, lysosomal enz, and
ROS
What produces RankL: t cells and synovial fibroblasts
Is there genetics in RA: yes: HLA-DR
Pannus consist of: inflammatory cells, granulation tissue, and
fibroblasts
Extra articular involvement in RA
o subQ rheumatoid nodules
o vasculitis and inf lesions in pericardium, myocardium, lungs,
pleura, peripheral nerves, and eyes
o systemic amyloidosis in some pts
o lab finding of hypergammaglobulinemia: RA
ass with gout:
o chronic hemolysis, polycythemia, leukemia, lymphoma,
diuretics, aspirin, nicotinic acid
o
gauchers dz, sle, or osteo radio necrosis after radiation: avascular
necrosis etiology
pale infarct or sequestrum and separtaiton of overlying cartilage
from bone: avascular necrosis morphology
avascular necrosis of the femoral head in children: legg calve
Perthes dz
JONES lymphoid neoplasm’s:
Part of LN where primary or secondary follicle (germinal center:
mantel zone) is: cortex
Part of LN where sinuses are located: medulla
Def: LN undergo reactive changes whenever they are challenged by
microbiologic agents, cell debris, or foreign matter introduced into
wounds or into circulation: lymphadenitis (NOT THE SAME AS
LYMPHADENOPATHY)
Acute or chronic no specific lymphadenitis:
o Nodes are swollen, grey-red and engorged: acute
o Distention of capsule causes tendernous to touch: acute
o Which one has three types: chronic
What are the three diff morphological alterations in
chronic non specific lymphadenitis:
1. Follicular hyperplasia
2. Paracortical lymphoid hyperplasia
3. Reticular hyperplasia.
What are the etiological factors in lymphoid neoplasm’s:
o Chromosomal translocations and oncogenes
o Inherited genetic factors
o Viruses
o Env agentes
o Iatrogenic
Whats the difference in leukemia and lymphoma
o Leukemia: use for lymphoid neoplasm’s presenting with
WIDESPREAD involvement of the bone marrow, usually with
presence of large number of tumor cells in the peripheral
blood.
o Lymphoma: used to decribe proliferations as DISCRETE tissue
masses.
What does the REAL chassification system use as the most
important criteria: immunophenotype
o Also: genetic aberrations
Precursor b cell neoplasm: neoplasm of immature b cell
Peripheral b cell neoplasm: neoplasm of Mature b cells
Precurso t cell neoplasm: neoplasm of immature t cell
Peripheral t cell and NK cell neoplasm: neoplasm’s of mature t/NK
cells.
Neoplasm’s of reed Sternberg cells and variant: hodgkins lympoma
What are the vast majority of lympoid neoplasm’s (80-85%): of b
cell origin
Nodular sclerosis and mixed cellularity seen in : V. Hodgkin
lymphoma
What is ALL: acute lymphoblastic leukemia/lymphoma
Most occur in kids under 15, 2x as common in whites, and more
frequent in boys than girls, and 85% are of precursor b cell tumors:
ALL
Abrupt stormy onset, bone pain and tendernous, generalized
lymphadenopathy, splenomegaly, or hepatomegaly and cns
manifestions: ALL
Lymphoblasts with scant cytoplasm and nuclear chromatin is
delicate and finely stippled: ALL
TdD is positive in >95%: ALL
blasts almosts always express the pan b cell molecule CD19/CD10:
ALL
How is early and late ALL distinguished: early doesn’t have
cytoplasmic IgM heavy chain ( mu chain)
35% or > # of blasts in the bone marrow is essential for dx: ALL
POOR prognosis Markers in ALL
o Age <2
o Presentation in adolescence or adulthood
o Peripheral blood blasts counts greater than 100K,
o Cytogenic aberrations such as t(9;22) (****the PHILADELPHIA
CHROMOSOME)
Favorable prognosis markers in ALL:
o Onset of dz between age of 2-10
o Low white count
o An early pre-B phenotype
The dx criteria is an absolute lymphocyte count > what ????? in
chronic lymphocytic leukemia/small lymphocytic lymphoma: >4000
per mm (cubed)
Most pts present at ages over 50 (median age is 60): chronic
lymphocytic leukemia/small lymphocytic lymphoma
Gender pref in chronic lymphocytic leukemia/small lymphocytic
lymphoma: M 2x
LN architecture is diffusely EFFACED by predominant pop of small
lympocytes 6-12 micro meters in diameter containing round to
slightly irregular neuclei with condensed chromatin and scan
cytoplasm: chronic lymphocytic leukemia/small lymphocytic
lymphoma
Smudge cells: chronic lymphocytic leukemia/small lymphocytic
lymphoma
B cel markers in chronic lymphocytic leukemia/small lymphocytic
lymphoma: CD19, CD20, CD23, CD5
Of the above which one is the t cell marker that is expressed on
only a small subset of normal b cells in chronic lymphocytic
leukemia/small lymphocytic lymphoma: CD5
Diffuse large b cell lymphoma transformation: Richter syndrome:
under chronic lymphocytic leukemia/small lymphocytic lymphoma
Heterogeneous group of tumor that constitute 20% of all NHL and
60%-70% of aggressive lymphoid neoplasm’s: diffuse large b cell
lymphoma
What is the median age for diffuse large b cell lymphoma: 60
This is fatal but easily treatable: diffuse large b cell lymphoma
Large cell (centroblastic) size (usually 4-5 x the diameter of a small
lymphocyte) and a diffuse pattern of growth: diffuse large b cell
lymphoma
What are the markers on b cell tumors in diffuse large b cell
lymphoma: CD19, CD20
Variable expression of germinal center markers CD10, and BCL6
and most have surface Ig, and all are negative for TdT: diffuse large
b cell lymphoma
African (endemic) Burkitt lymphoma
Sporadic (nonendemic) Burkitt lympoma
Faintly basophilic or amphophilic cytoplasm: burkitts lymphoma
Mitotic figures are numerous and evidence of APOPTOSIS: burkitts
lymphoma
Starry sky pattern: Burkitts lymphoma
Tumor cells are mature b cells expressing surgace IgM, monotypic
kappa or lambda light chain, CD19 CD20, CD10, and BCL6: Burkitts
lymphoma
All forms of burkitts lymphoma are ass with the translocation of
the :c-myc gene on chromosome 8
What age does most Burkitts lymphoma affect: children and young
adults
Mass involving the mandible, abdominal viscera particularly the
kidney, ovaries, and adrenal glands: Burkitts lymphoma (endemic
form)
Most often presents as ab mass involving ileocecum and
peritoneum: Burkitts lymphoma (sporadic form)
Prognosis of BL: good when tx with high dose chemotheraphy for
short term
Another name for multiple myeloma: plasma cell myeloma
The most imp and most common symptomatic monoclonal
gammopathy: multiple myeloma
Gender preg in multiple myeloma: men
High serum levels of IL-6 are ass with poor prognosis; multiple
myeloma
Most often affects the verterbral column: multiple myeloma
The bone lesions appear as punched out defects 1-4 cm on xray:
multiple myeloma
Flame cells: multiple myeloma
Mott cells: multiple blue grapelike cytoplasmic droplets and cells
containing variety of other inclusions such as fibrils, crystallien rods,
and gobules sometimes russell bodies (cytoplasmic) or dutcher
bodies (nuclear): multiple myeloma
Rouleaux formation caused by high levels of serum M proteins in :
multiple myeloma
99% of pts with show increased levels of immunoglobulins in blod
and or light chains in urine known as BENCE JONES PROTEINS:
multiple myeloma
most common serum monoclonal immunoglobulin (m protein) in
multiple myeloma: IgG
prognosis of multiple myeloma: variable but generally poor
high incidence in adults infected by HTLV-1: adult t cell
leukemia/lyphoma
hypercalcemia: adult t cell leukemia/lyphoma
cells have multilobulated nuclei called cloverleaf or flower cells;
adult t cell leukemia/lyphoma
prognosis of adult t cell leukemia/lyphoma: death within 1 months
to 1 year even with aggressive chemo
mycosis fungoides progresses to : Sezary syndrome
different manifestations of a tumor of cd4 helpter t cells
characterized by a marked predilection to involve the skin.: mycosis
fungoides/Sezary syndrome
shows three distinct stages of
o 1:inflammatory premycotic phase,
o 2. Plaque phase
o 3. Tumor phase
Answer: mycocsis fungoides
Manifests as generalized exfoliative erythroderma: Sezary
syndrome
o
o
Neoplastic giant cells seen in HL: reed Sternberg cells
What are reed Sternberg cells derived from in HL: from germinal
center or post germinal center b cells
Most common form of malignancy in young adults with ave age of
dx of 32: HL
Prognosis of HL: curable in most cases
Nodular sclerosis pts usually present as: stage 1 or 2 M=F
Mixed cellularity pts usually present as: state 3 or 4 M>F
Lacunar cells: type of reed Sternberg cell seen in HL
Mummification: HL
Spread of HL: nodal dz firstsplenic dz hepatic finally marrow
and extranodal involvement
Most common form of HL: nodular sclerosis type
Nodular type most common cell type: lacunar cell type
Collagen bands: nodular type of HL
CD15 CD30 and neg for CD45 and b cell and t cell markers: nodular
type of HL
Which type of HL has strong ass with EBV: mixed cellularity type
Which has best prognosis: nodular type
Which one has night sweats and weight loss: mixed cellularity type
Lytic lesions: Multiple myeloma
Mott cell: multiple myeloma
\
HEME OLD TEST QUESTIONS
Common translocation is the IgH locus t(8:14) but there may also be
t(2;8) or lambda (8;22) light chain locus: burkitts lymphoma
Jaw involvement: endemic burkitts lymphoma
Philadelpha chromosome: poor prognosis in ALL
Majority of cells have terminal deoxynucelotideyltransferase (TdT)
(+ in >95%) of cases): ALL
Absolute lymphocytic count >4000 per mm cubed: CLL
Intranasal cyanocobalamin for : tx fof vit b12 def
Inhibits dehydroooratic acid dehydrogenase: luflunamide (arava)
Smudge cells: CLL
In the anemia, RBC hemolysis results in
o Increased internal viscosity
o Hb aggregation
o Decreased cell water
o And precipitated hb as Heinz bodies:
Answer: G6PD def
What can cause G6PD def to unmasked:
o Exposure to some drugs: ex sulfonamide
o Inf
o Diabetic acidosis
o Neonatal period
o Ingestion of FAVA beans
Fava: g6pd def
Osmotic fragility: hereditary spherocytosis
Whats the tx for hereditary spherocytosis/elliptocytosis:
splenectomy
Defect in the RBC membrane results in these two disease:
hereditary spherocytosis and hereditary elliptocytosis
Found in southern japan, west Africa, and Caribbean basin: adult T
cell leukemia/lymphoma
Starry sky appearance: burkitts lymphoma
In an indirect coombs test where is the ab: in the plasma
In a direct coombs test, where is the ab: on the membrane
Hematuria vs hemoglobinuria: in hematuaria the rbc is intact where
as in hemoglobinuria the rbc is lysed.
Which one of the above is ass with uti or kidney problems:
hematuria.
Which one of above is associated with hemolytic anemia:
hemoglobinuria
Polycythemia aka: erythrocytosis
In relative polycythemia due to dehydration, hct levels are: high
In relative polycythemia due to spurious, hct levels are low
What are the macrocytic anemia: (mcv <80)
o Find those small cells henry
Fe def or pb poisoning
Thalassemias
Sideroblastic (80%)
Chronic dz
Hemoglobinopathy
What is the purpose of a reticulocyte count: it shows how fast rbc
cells called reticulocytes are being made and put into the blood.
o The reticulocyte count can rise in dz where there is a loss of
blood or premature destruction of rbc such as in : hemolytic
anemia
Petechia: platelets
What are the four d’s of quantitative thrombocytopenia:
o Increased destruction
o Decreased production
o Dilution
o Distribution
What are the myeloid cells:
o Basophils
o Rbc
o Eosinophils
o Monocytes
o Platelets
o
What are the lymphoid cells
o Lymphocytes
o Lymph nodes
o Extranodal tissue
Myeloproliferative disorders affect what age: adults
Myeloproliferative disorders are disorders of: stem cells
What does the peripheral smear show in Myeloproliferative disorder:
increased platelets
What does the bone marrow show in myeloproliferative disorders:
HYPERcellularity
Myeloproliferative disorders:
o Name the predominant phenotypes
Chronic myeloid leukemia: granulocytes, basophils,
eosinophils
Polycythemia vera: erythocytes
Essential thrombocythemia myelofibrosis:
platelets/megakaryocytes
Chronic myelomonocytic leukemia: monocytes
Walenstroms macroglobulinemia: plasma cell disorder
What are all the plasma cell disorders
o MM
o LIGHt/heavy chain dz
o Benign monoclonal gammopathy
o Waldenstroms macroglobulinemia
o Amyloidosis
o Plasmacytoma
o Plasma cell leukemia
o Monoclonal gammopathy of undertermined significance
How to evaluate coagulopathy
o Whats the best way: Hx and PE
o CBC with smear
o PT: prothrombin time
Extrinsic pathway: factors 2, 5, 7, 10 and fibrinogen
o INR: international normalized ratio
Ratio of pts PT to normal PT
o PTT: partial thromboplastin time
Factors: 8,9,11,12, and rarely von villebrands factor
o Platelet count
What are the inherited defects in anti-clot enz ass with
hypercoagulable states: protein C and protein S def.
o Also (HIT ab)
What are the clinical manifestations of anemia (TQ 100%)
o Fatique/weakness
o Dizziness/ syncope
o Intermittent claudication
o Muscle cramps at night
o HA/ lightheadedness
o Angina
o Tinnitis
What is on a peripheral smear in hemolytic anemia: spherocytes
TQ 100%: general lab features of hemolytic anemia
o Low Hgb/Hct
o HIGH reticulocyte count
o Increased LDH
o DECREASED haptoglobulin
o Increased bilirubin
What are the SPECIFIC lab features of hemolytic anemia
o Schistocytes/poikliocytes/spherocytes/ovalcytes/sickle cells
o Hemoglobin electrophoresis
o Hemoglobinemia/hemoglobinuria
o Osmotic fragility
o DAT/IAT/ eluates
o G6PD level
o ETC
Priapism: perpetual erection seen in sickle cell
What is the most commonly used anti-sickling agent tx for sickle
cell: hydroxyurea
Which one do you use an RBC exchange: sickle cell anemia
Beta thal minor: thal triait: 1 gene abnormal: normal hb
Beta thal intermeida: 2 genes abnormal but some beta globin
produced: mild to moderate anemia
Beta thal major: 2 genes abnormal and little beta globin produced:
severe anemia: high Hgb F -1 to 5% HgbA
Alpha thalassemias
o Silent carrier: 1 missing gene, normal Hgb: no dz
o Alpha thalassemia trait/alpha thalassemia minor: 2 missing
genes: mild anemia
o Hemoglobin H: three missing genes mod to severe anemia
o ***** it takes three lines to make an H)
o alpha thalassemia major or hydrops Fetalis: all Four genes
missing: death before or shortly after birth
tx is none except transfusion and IRON Chealation with BAL:
thalassemias
Heinz bodies: eating fava beans in G6PD def
Whats does WAIHA stand for: warm autoimmune hemolyic anemia
Is warm ab auto or Alloimmune: autoimmune
Is HDN auto or allo: Alloimmune
Penicillin: Alloimmune
Lupus is ass with : autoimmune
What are the lab values in hemolytic anemia
o Anemia
o In vivio hemolysis
o +DAT
o + IAT
o eluate to ID antibody
o reticulocytosis
o spherocytes
o splenomegaly
o
what is the antibody in WAIHA: IgG
TTP is a type of : acquired hemolyic anemia
What is the pentad of TTP
o Microangiopathic hemolytic anemia
o Fever
o Renal failure
o Mental status changes
o Thrombocytopenia
Adempts 13 def: TTP
Tx is plasma exchange: TTP
What is the disorder in AA: the pluripotent stem cell
What are the hallmarks of AA:
o Requires both
Pancytopenia
Acellular bone marrow filled with fat
What is the cuase of AA: stem cell injury
What are you looking for on AA bone marrow bx: spicules with
empty fatty spaces
What is the total lab eval in AA
o Pancytopenia
o Acellular or hypocellular BM
o Decreased CFU-GM
o Decreased BFU-E
o Decreased Long term culture initiating cells to les than 1%
o Flow cytometry rules out ALL or hairy cell leukemia
What rules out ALL and hairy cell leukemia in AA: flow cytometry
Tx for AA: bone marrow transplant
What test to order to r/o myelodysplastic syndrome: cell
morphololgy/ cytogenetics, cell surface markers
Test to r/o PNH: + sucrose hemolysis test/ abnormal CD59
ALL: cell surface markers/marrow
Hairy cell leukemia: cell surface markers and special stains
Kimmel last days before exam
Paste features seen in what: generalized discoid lupus (DLE)
What does paste stand for
o Follicular Pluggin
o Epidermal Atrophy
o Scale
o Tenangiectasia
o Erythema
Which type of cutaneous lupus has the greatest risk for SLE (70%):
acute LE (ALE)
What are the possible involved areas in systemic lupus (SLE): old TQ
o Skin, joints, kindneys, lungs, brain, heart, blood forming
organs
Photosensitivity and malar rash: SLE
What are the hematological levels in SLE:
o Hemolytic anemia
o Leukopenia: <4K
o Lymphopenia: <1.5k
o Thrombocytopenia: <100K
Pt with the following: ds dna, sm, antiphospholid with false + VDRL,
cariolipin ab, and lupus anticoagulant: SLE
If you get a neg ana for sle: much less likely that you have SLE
If you get a + ana what is next step: further testing
What are the major specific serologies for SLE
o DS DNA
o ENA
o ACL abs
What is the sensitive but not specific serology for SLE: ANA
What do you tx the skin in sle with:
o Antimalarials
o Steroids
o Dmards (anti metabolites, MTX, Leflunomide, MMF, aza)
o Cytotoxic: CSA, CTX)
What do you tx the joints in SLE
o NSAIDS: only if no kidney dz
o Anti malarials
o Steroids
o Dmards
o Biologicals
Major inter organ tx in sle:
o High dose steroids
o +/- cytotoxic agent
for SLE kidney tx
o focal proliferative : steroids
o diffuse proliferative: steroids + Cytoxan/MMF or BM transplant
o membranous: steroids +/- IMURAN/ cyclosporin A
what do you tx the thrombocytopenia in SLE with if its
o mild: Plaquenil or danazol
o severe: prednisone (others in notes)
what is usually not affected in drug induced lupus: major internal
oragn dz or DNA abs
TQ: what are the drugs that can cause drug induced lupus
o P(2x)HIC Q
Procainamide
penicillamine
Hydralazine
Isoniazid
Chlorpromazine
Quinidine
Increased PTT time not corrected by mixing study:
anti=phospholipid ab syndrome
What is the tx of the above: anticoagulant
Cardiolipin ab: anti-phospholipid ab syndrome
What things can be false + in pt takig anticoagulants in anti-
phospholipid syndrome: lupus anticoaguland and PTT time
Tx in anti phosopholid ab syndrome for pts with previous
thromobotic episodes: Warfarin with INR
Morphea: localized scleroderma
Crest: limited cutaneous scleroderma
Pulmonary HTN: limited cutaneous scleroderma
SCL-70: diffuse cutaneous scleroderma
Cancer risk such as lung cancer in: scleroderma
Manifestions such as raynauds, digital ulcers, calcinosis, MSK, GI,
renal, pulm, and cardiac : scleroderma
Tendon inflammation/ friction rub/tenosynovitis in: PSS
Ace inhibitor or vasodilator: tx option for PSS
Name the dz: symmetric proximal muscle weakness, elevated
Muscle enz such as (CPK, aldolase, and transaminase, LDH),
myopathic EMG abnormalities, typical changes in muscle bx,
heliotropic rash: polymyositis and dermatomyositis
What are the ab ass with dermatomyositis: JO-1, PM-1
If you have myositic what tests should you have: age related
malignancy eval on first dx + CXR and CT scan abdomen/pelvis and
yearly thereafter
Antibody for Mixed CT dz: anti ribonuclear *anti RNP
Ditcheks lectures
Best for spatial resolution: plain radiograph
Initial for trauma: plain radiograph
Good for pre-opt: CT
High contrast resolution:MRI
No radiation and used to differ btw cystic and solid lesion:
Ultrasound
Avascular necrosis (AVN): MRI
Achilles tendon: ultrasound
Foreign body such as WOOD: Ultrasound
Advantage is a head to toe scan: bone scan
What is bone densitometry (DEXA) used for : osteoporosis
T scoring:
o Normal: within 1
o Osteopenia: 1-2.5 below the young normal bone density
o Osteoporosis: >2.5 below
Def of comminuted: >2 fragments
Impactation: bone teloscopes into itself
Pathological fx: through abnormal bone such as in pagets dz
Lipohemarthrosis: fat and blood in joints: means there is a crack in
the blood and the BM fat and blood leaked out.
Pain and tendernous on outstretched hand: scaphoid fx
For knee trauma to visualize ligaments, tendons, and cartilage such
as PCL or meniscus tear: MRI
Best for comminated fx: CT
Ped fx vs adult: peds bones more water content so they bend before
breaking. Can result in bent bone
Buckling on xray: torus fx
Bowing fx: from microfractures
Look at salter harris classifications: TQ 100%
Exits through metaphysis: type II
Exits through epiphysis: type 3
Exits through both: type 4
Fat pads in elbow: elbow fx
o You should never see a posterior fat pad. Maybe you could
see a anteriorly normally
Posterior rib fx: child abuse
Bucket handle or corner fxs: child abuse
Multiple fx in varying stages of healing
What are the ABCDS of arthritis
o Alignment
o Bone mineralization
o Cartilage (joint space)
o Distribution
o Soft tissues
Ulnar deviation and periarticular osteopenia: RA
Joint space narrowing and erosions: RA
Young males, proximal large joints (spine/SI joint), and bamboo
spine: ankylosing spondylitis
Non erosive, subluxations, distribution similar to RA (MCP) joints:
SLE
First MTP: podagra: gout
Distal joints in hands and erosive and productive and SI joints/spine
involved: psoriatic arthritis
Which is the only one without erosions: SLE
TQ: signs of non aggressive lesions
o Narrow zone of transtition
o Benign patterns of periosteal rxn
Thick
Wavy
Dense
o No cortical destruction
TQ: signs of aggressive
o Wide zone of transition
o Permeative or moth eaten lytic pattern
o Aggressive periosteal rxn
Sunburst, onion skin, codmans triangle
o Cortical destruction
Monostotic: one location
Which test to see monostotic vs polyostotic or increased bone
turnover: bone scan
Soft tissue mass: MRI
Rupture of rotator cuff: MRI
Metastatic breast cancer: CT scan or MRI
Evaluation pros for nuclear med for osteomyeltitis
o High sensititivity (bone turnover)
o Detects earlier than plain radiograph
o No artifact from prosthesis (unlike mri)
Cons:
o Low specific
o Low resolution
o No info on soft tissues such as abscesses
Best for osteomyeltits marrow edema: MRI
Weisberg
Of the hodgkins which is more common: NHL
Viral genomic material is found in 50% of HL tumors: EBV
What is the gender preg for HL: Male
Is HL genetic: Yes
Painless lymphadenopathy, granulocytic leukocytosis with
eosinophila: HL
What is the alkaline phosphate level in HL pts: elevated
What are the B symptoms: Fever, night sweats, weight loss more
than 10% total body weight
What cell origin is HL: B lymphocytic origin
Immunoperoxidase rxn in HL will detect what two antigens helpful in
dx HL: Leu-M1 (CD15) and Ki-1 (CD30)
What is the most common type of HL: nodular: also this is the best
prognosis also
o COTSWORLD STAGING CLASSIFICATION
Waldeyers rings: stage I
2 or > and on same side of diaphragm: II
what is the consistency in HL lymph nodes: rubbery
what does X mean: bulky dz
o greater than 1/3 widening of mediastinum
o greater than 10 cm dimension of nodal mass
E stands for : involvement of a single extranodal site
Unfavorable prognosis factors of localized dz (stage I or II)
o Elevated ESR >30
o Histology: mixed cellularity or lymphocyte deplated
o Bulk dz: ratio of mediastinal mass to thoracid diameter >1:3
o 4 sites on same side of diaphragm
what are some unfavorable prognosis factors in advanced dz
o serum albumin <4g
o Hb <10.5
o Male
o Stage IV
o WBC >15,000
o Age >26
o Lymphocyte count <600 or <8% of white count
what makes pain worse in HL when drinking it: alcohol
tx for stage IA or IIA
o radiation and often preceded by brief chemo
what are the long term effects of radiation therapy
o HYPOthyroidism
o Radiation fibrosis
o Effusions
o Late occurring solid tumors in field of irradiation
o Sterility
What is the tx for advanced age dz of HL (IIIA, IIIB, or IV)
o Combination chemotherapy as primary therapy with the
possibility of radiation for residual masses
In the above what does combonation therapy consists of
o MOPP
Mustargen
Oncovin
Prednisone
Procarbazine
o Or ABVD---------------->>>> first line now
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
What are the drugs in the HIGH dose therapy options for pt that
relapse
o CBV
Cyclophosphamide
Carmustine
Etoposide
o BEAM
Carmustine
Etoposide
Cytarabine
Melophalan
NON HODGKIN LYMPHOMA
What are the cell types involved: B cell, T cell or NK lymphocytes
What is the tx of choice for NHL: Rituxan
Low grade lymphomas in NHL are: seldom curable
o But tx can often be delayed with no consequences
TQ: what are the infection agents knows to cause NHL
o EBV
o HTLV1
o H. pylori
What are the physical or chemicals ass with NHL
o Diphenylhydantoin
o Dioxin
o Solvents
o Radiation
o Prior chemo
o Prior radiation
Inherited immunodeficiency dz’s
o Klinefelters
o Chediak higashi syndrome
o Ataxia telangictasia syndrome
o Wiscott Aldrich syndrome
o Common variable immunodef syn
Acquired immunodeficiency dz
o Iatrogenic immunosuppression
o Acquired immunodeficiency syn (HIV)
o Acquired hypogammaglobulinemia
Autoimmune dz
o Sjogrens
o Non tropical sprue
o RA
o SLE
What are the mature (peripheral) b cell neoplasm’s
o B cell lymphocytic leukemia/small lymphocytic lymphoma
o Follicle center lymphoma, follicular (most common)
o Mantel cell lymphoma
o Diffuse lare cell b cell lymphoma
o Burkitts lymphoma/Burkitt cell leukemia
What are the precursor t cell neoplasm’s
o Precursor t lymphoblastic lymphoma/leukemia
What are the mature (peripheral) t cell and NK cell neoplasm’s
o Mycosis fungoides/Sezary syndrome
What is the most common site of MALT lymphomas: stomach
What are the causes of gastric MALTomas: H pylori thus (TQ) you
can tx MALT with an antibiotic
Whats the t cell ones: mycosis fungoides and Sezary syndrome
Localized vs advanced
o Localized (must have all three)
1. Ann arbor I or II
2. No tumors >10cm
3> no B symptoms
o advanced (May include any of the three)
1. Ann arbor III or IV
2. Tumors >10cm (bulky dz)
3. B symptoms present
explain the IPI pts
o APELS
Age over 60: 1 pt
Performance exam > or = 2: 1 pt
Extranodal sites >1: 1 pt
LDH above ave: 1 pt
Stage III or IV: 1 pt
What is the difference in the FLIPI index
o The number of LN involvement has to be 4 and HB <12.
Tx for indolent lymphomas
o Alkylating agents: chlorambucil, cyclophosphamide,
bendamustine
o Anthracyclines: doxorubicin
o Purine analogues: fludarabine
o RUTUXAN: chimeric ab with anti CD20 activity
o
What are some signs of progression to aggressive lymphoma in NHL
o New or worsening B symptoms
o Facial or UE swelling
o Ab pain
o Jaundice
o Constipation
o Bowell obstruction
o Painful, rapidly enlarging LN
o Tender splenomegaly
o Superior vena cava syndrome
o LE edema
o Thrombocytopenia
o Granulocytopenia
o Rising serum creatinine
o Hyperbilirubinemia
o Hydronephrosis on CT
What are the chemotherapy tx for NHL
o Single alkylating agent
Chlorambucil, Cytoxan
CHOP, CVP
New agents: bortezomide, bendamstine,
radionucleotides
Intravascular hemolysis anemias
o Hemolytic anemia
Hemoglobinemia and hemoglobinuria
Jaundice
FEMALE preference
RA
Juxtacortical osteosarcoma
Giant cell tumor of bone (osteoclastoma)
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