Hemotologic DisordersBy Diana Blum RN MSN
Metropolitan Community CollegeNURS 2140
Assessment
Should be subjectiveShould be objective
Anatomy
Blood is a type of connective tissue◦Transports nutrition ◦Transports oxygen◦Transports hormones
Immunological aspects also important◦Critical to body defense
◦Maintains temperature◦Controls PH◦Removes toxins◦Regulates electrolytes
Hematopoiesis:
Bone Marrow:
Stem Cell:
Cell Types (page 1991-94)
Plasma:
Leukocyte:
Reticulocyte:
WBC:
RBC:
Erythrocyte:
Platelet:
Lymphatic system
Responsible for removing excess fluid protein and large particles from cells
Main functions: ◦fluid balance maintenance◦Defense◦Fat absorber
Spleen
Stores 20-40 ml bloodRemoves old cellsFilters and destroys antigens before they
enter systemStores platelets (1/3 of body’s store)
Hemostasis (page 1995)
Stopping of blood flow2 phases:
◦Primary◦Secondary
Critical thinking here
Be able to explain tests to ptsBe able to explain why tests being doneKnow normal from abnormalBe good communicator with physicianBe able to consider anticipated actions
◦Ex. When blood transfusion needed
Anemia (page 2008)
Hemoglobin is lowOxygen is low Not a disease but an underlying condition2 types: hypoproliferative and hemolytic
◦Hypoproliferative: marrow can’t produce adequate erythrocytes Lack of factors, chemicals, marrow damage
◦Hemolytic: premature destruction of erythrocytes. Leads to tissue hypoxia Abnormality like sickle cell, direct injury
Erythropoiesis
Stimulation of RBCs by erythropoietin
Erythropoietin: hormone produced by kidneys during periods of hypoxia to ensure growing tissues will have sufficient oxygen.
Determination between types
Based on:◦Marrow ability to respond to decreased
erythrocytes ◦Manner of maturity for young erythrocytes◦The presence or absence of end products of
erythrocyte destruction within the circulation
Clinical manifestations (pg 2010)
Rapidity with the anemia has developedDuration of anemiaMetabolic requirements of patient Other disorders
Cardio: tachycardia, hypoxia, MI symptomsRespiratory: SOB in varying degreesNeuro: headache, poor concentration, vertigo,
irritibility, confusion
Compensation also may occur
Education
Prioritize activitiesPlan rest periodsEats healthy foods rich in iron, vitamins, and
proteinsUse nutrition supplements as neededReport palpitations, dizziness, dyspneaMonitor vitalsMonitor edemaMonitor neuro statusSafety techniques
Low RBC Low HGB andHCT Low serum Iron level Low ferritin level High TIBC level Results from diet low in iron Results from body not absorbing enough iron from GI tract
◦ Not enough hemoglobin made as result s/s: sore tongue, brittle nails, fatigue, pallor, orthostatic changes (in
severe cases) Dx: bone marrow aspiration, lab work Tx: iron supplements, iron rich foods (liver, oysters, red meat, fish,
dried fruits, legumes, dark green veggies, whole grain breads and cereal)---orange juice or other vitamin C product will enhance iron absorption◦ What is the major side effect of IV iron?
Iron Deficiency Anemia
Anemia and Renal Disease
Pts don’t become severely anemic til creatinine levels exceed 3mg/100ml
Hematocrit decreasesCauses:
◦shorted RBC lifespan◦Deficiency of erythropoietin◦Long term hemodialysis
Tx: monitor serum levels of iron, hematocrit, and vitals
Anemia and chronic disease
Only inflammation, infection, and malignancy cause
It is nonprogressiveDevelops over 6-8 weeksMany don’t require treatment
Most cases are idiopathic Infection and pregnancy, medication, chemicals or radiation
can trigger◦ Airplane glue, arsenic, pestisides
Complete failure of the bone marrow Low RBC count Low WBC Low Platelet cts S/S: pallor, fatigue, tachycardia, sob, hypotension,
prolonged/spontaneous bleed, frequent infections, purpura, retinal hemorrhages are common
Tx: transfusion of RBC and Platelet, antibiotics, corticosteroids, bone marrow transplant, ICU setting, monitor for signs and symptoms of infection and bleeding, neutropenic precautions
Aplastic Anemia
Pt does not absorb vitamin B12 from stomach◦ Higher incidence of gastric cancer
Pt may lack intrinsic factor-essential for b12 absorption Assess hx: gastrectomy, crohn’s, family history,etc. s/s: weakness, sore tongue, numbness of hand and
feet Tx: B12 injections monthly, endoscopy q1-2 years---if
untreated can develop heart failure and lead to death◦ Monitor oral cavity, and skin. Monitor for jaundice, monitor
gait, provide small frequent bland diet Dx: Obtain shilling test
Pernicious Anemia
Thalassemia
Most frequent in SE Asian, African, and Mediterranean descent
Folic Acid Deficiency
Folate is in green leafy veggies and liverAlcohol increases need for folic acidS/S same as pernicious anemia except no
neuro symptomsDx: folate levelTx: replacements
RBCs normally disc shaped In sickle cell they are sickle shapedEasily ruptureObstruct blood flowGenetic in natureMost common in African AmericansSickle cell is recessive (inherit from mom
and dad)
Sickle cell anemia
Sickle Cell Crisis
3 types: ◦sickle crisis: most common---obstructive◦ aplasic crisis---from infection from parvovirus◦ sequestrian crisis---result from other organs pooling
sickled cells Spleen is most common Also in liver and lungs
S/S: tachycardia, fever, decreased HGB, infiltrates on chest x-ray
Pulomary HTN is a symptom that is not usually detected until damage is irreversible
Monitor x-rays, CT scan, Echo, HGB levelTx: treat symptoms, Bone marrow transplant,
transfusions, Hydroxyurea, Arginine
Glucose -6-Phospate Dehydrogenase Deficiency
This is a deficiency in the gene that is essential for membrane stability
Hemolysis only occurs with stressful situationsMostly affects african americans, greek or italian
decent as well as asians and jewish origins Inherited on x chromosomeS/S: asymptomatic, or jaundice, pallor,
hemoglobinuriaDx: serum G-6-PDTx: stop offending med, transfusion, educate about
meds to avoid, instruct to wear medic alert bracelet
Enough RBCs made but they are destroyed once they are released into circulation
Causes: infection, drug reaction, cancers/s: pallor, fatigue, tachycardia, sob,
hypotension, jaundice, high bilirubin levelsPositive direct coombs antiglobulin testTx: blood transfusions, corticosteroids,
spleenectomyRecovery in few days to weeks
Autoimmune Hemolytic Anemia
Too many RBCs produced◦ Blood more viscous
s/s: headache, dizziness, ringing in the ears, blurred vision, ruddy complexion, HTN, pruritis, gout problems, burning fingers and toes
DX: o2 sat, CBC with DIFF Complications: CVA, MI, BLEED issues Tx: hydroxyurea, interferon, plavix, ASA Treat symptoms Teach pt: don’t cross legs, quit smoking, stay active, minimal
alcohol, avoid excess iron phlebotomize blood
Use 18 gauge needle In antecubital vein and remove I unit of blood
Polycythemia Vera
Leukemia
Defect in the WBC stem cellAbnormal reproductionsUnknown cause, may be from chemical or
radiation exposureSymptom onset is abrupt
AMLAcute Myeloid Leukemia
Defective stem cell Incidence rises with age 60+Prognosis variableYounger pts may survive 5 yearsS/S: fever, infection, weakness, fatigue, bleed
tendency, painDx: no warning, CBCComplications: bleeding (petechiae, ecchymosis),
infection, DICTX: chemo, transfusions, infection treatment, growth
factor agents, bone marrow transplant, palliative care
CML
Uncommon in pts under 20Life expectancy 3-5 yearss/s: asymptomatic, dyspnea, mild
confusion, enlarged liver, wt loss, anorexiaTx: tyrosine kinase inhibitor, avoid
antacids and grapefruit juice, interferon, cytosine, leukopheresis, bone marrow transplant
Acute Lymphocytic LeukemiaALL
Most common in young kids 4-14Uncommons/s: decreased cbc counts, pain, enlarged
liver, enlarged spleen, headache, vomitingTx: bone marrow transplant, chemo,
corticosteroidsComplications: infection
CLL
Common in older adultsMost common form in US and Europe More frequent in mens/s: asymptomatic, lymphadenopathy,
pain, hepatomegaly, spleenomegaly, anemia, fevers, drenching sweats, wt loss, infections
Tx: treat symptoms, chemo, monoclonal antibodies
Hodgkins Disease
Characterized by reed- sternberg cells in the lymph nodes
Highest occurance is in 20s and50sMen are more likely than women to haveTx: radiation, chemo, bone marrow
transplant, stem cell transplantSurvival rates vary5 yr survival rate is 82%
Non Hodgkins Lymphoma
6th most common type of cancer and cancer death in US◦Avg age of dx is 60
Stages◦Low grade◦ Intermediate grade◦High grade
The higher the grade the more aggressiveTx: chemo, radiation, bone marrow transplant,
stem cell transplant5 year survival rate is 52%
Multiple Myeloma
Cancer of the plasma cellsMost common over the age of 60No known cause
◦Genetics and radiation exposure play a parts/s: bone pain, hyperuricemia (kidneys), anemia,
hypercalcemia, fractures, spinal cord compression, renal failure
Diagnosis: radiographs, serum and urine protein electrophoresis, bone marrow biopsy
No known cureTx: chemo and radiation to treat symptoms,
vertebroplasty, antiemetics, pain management
Bleeding disorders
Clotting Cascade
http://www.mhhe.com/biosci/esp/2002_general/Esp/folder_structure/tr/m1/s7/trm1s7_3.htm
Too few platelets◦Causes: cancer treatment
Too many platelets being destroyed◦Causes: idiopathic thrombocytopenic purpura,
thrombic thrombocytopenic purpuraDx: bone marrow bx, CBC s/s: petechiae, purpura, gingival bleed,
epistaxis, prolonged bleeding Tx: transfusions, spleenectomy, chemo, stop
med that is causing, stop ETOH, treat infection
Thrombocytopenia
Immune thrombocytopenic purpura
Most common among kids and young girlsUsually 1-6 wks after viral infectionCause unknown, h. pylori may play roles/s: asymptomatic, low platelet count, petechiae,
hemoptysisDx: bone marrow bx, platelet ctTx: stop med, immune globulin, chemo,Nsg: assess lifestyle, teach symptoms, avoid
constipation, avoid valsalva, avoid teeth flossing, use only electric razors, use only soft bristled tooth brush, monitor for osteoporosis
HIT
Example of drug induced immune mediated thrombocytopenia
RareRelated to heparin therapyTx: observation, assessment, monitor lab
values
Von Willebrand’s Disease
Most common inherited bleeding disorder1-2% international populations/s: Mucosal and cutaneous bleeding,
porlonged bleed times
Tx: DDAVP, FFP, Cryoprecipitate, bleed precautions
Thrombic Thrombocytopenic Purpura
GeneticLack of blood clotting factor1-2 cases per 20,000 personsTypes
◦A: factor VIII is missing-higher incidence◦B: factor IX is missing
Trait is carried on x chromosomeRare for women to haves/s: uncontrolled bleed especially in joints, skin, GI
tractTx: no cure. Transfusions, pain treatment (IV
morphine is common)..monitor for addiction to opiods.
Hemophilia
DIC
Not a diseaseTriggers: sepsis, trauma, cancer, shock, abruptio
placentae, allergic reactionsLife threatening conditionInitially coag time is normalS/S: are manifested in organs with either clots or
bleeding. Bleeding from all orifaces(tear ducts, gums, IV sites, rectum, urethra, etc.)
Dx: PT, PTT, D-DimerTx: treat the cause, replace fluids, o2, fix electrolyte
imbalances, administering vasopressors are vital, cryoprecipitate to replace factor 5 and 7, FFP, heparin
Universal donor= O-◦Does not contain A, B, or Rh antigens
Universal recipients= AB+◦Blood contains A, B, and RH antigens
Usually blood banks exactly match the pt blood
Blood typing for transfusion
Pt needs 18 or 20 gauge IV needle so cells are not lysed (destroyed)
Prior to administration, blood needs to be checked by 2 licensed nurses. Check the expiration date, name, medical record number, type of blood, blood band id, pt birthday◦Check vitals prior to administration
**blood must be initiated with in 30 minutes of arrival from lab to floor
Use blood tubing for administrationMonitor for blood reactionsMonitor vitals continuously during administration
adminstration on blood
Hemolytic: fever, chills, nausea, dyspnea, chest pain, back pain, hypotension◦ Antigen/antibody rx to transfusion◦ Happens shortly after initiation◦ Tx: stop the transfusion, call md, supportive therapy to maintain HR and BP
Anaphylactic: urticartia, wheezing, dyspnea, hypotension◦ Type 1 hypersensitivity rx to plasma proteins◦ Occurs within 30 minutes of initiation◦ Tx: stop transfusion, call md, be ready for epi and steroids
Febrile: fever, chills◦ Recipients antibodies rx to donor leukocyte◦ Occurs within 30-90minutes of initiation◦ Tx: stop infusion, call md
Circulatory overload: cough, frothy sputum, cyanosis, decreased BP◦ cardio system is unable to manage the additional fluid load◦ Occurs anytime during transfusion and up to several hours after completion◦ Tx: stop infusion, call for help, be prepared for code, be prepared to administer oxygen and Lasix
Blood reactions
Minimize the # of invasive procedureAvoid prolonged tourniquet useAvoid IM injections Instruct the client to use soft bristled
tooth brushNo strait edge razor shaving only use
electric razorAvoid NSAIDS
Bleeding precautions
Risk for injury r/t bleed. Goal: cessation of bleeding aeb no visual signs of bleeding and stable vs
Acute pain r/t bleeding into closed spaces(creating pressure on nerves. Goal: pain relief aeb: patient states pain is relieved and appears to be in relaxed manner.
Ineffective therapeutic regimen management r/t lack of knowledge about dx process and self care. Goal: effective management of condition aeb patient accurately describes condition and demonstrates self care measures.
Nrsg diagnosis
ANY QUESTIONS???