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M.G IN

ORAL

MEDICINE

2013-2014

Hematologic Diseases 18 Pages


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M.G IN ORAL MEDICINE | Hematologic Disease

1Hematologic Disease

HematologicDisease

Red Blood CellDisorders

Polycythemia

Erythrocytosis

Absolute

Primary

Secondary

Relative

Anemia

Etiology

Blood Loss

DecreasedProduction

IncreasedDestruction

Size

Microcytic

Normocytic

Macrocytic

Conc. of Hb

Hypochromic

Normochromic

White BloodCell Disorder

QuantitativeDisorder

QualitativeDisorder

Hematologic Diseases

Red blood cell disorder

A) Polycythemia = Erythrocytosis Erythrocytosis describes conditions with an increase in circulating red blood cells (RBCs),

characterized by a consistently raised hematocrit (HCT)

It is classified intoa) Absolute Erythrocytosis (true increases in red cell)b) Relative Erythrocytosis (normal red cell, reduced plasma volume)

Absolute Erythrocytosis are usually subdivided:a) Primary: excessive proliferation of erythroid elements independently of extrinsic

influences or by responding inadequately to them. (e.g. polycythemia Vera)

b) Secondary: increased erythropoietin production to compensate for hypoxia, (may becaused by lowered oxygen tension as; High altitude, Heart disease, Pulmonary

disease).


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Primary (Polycythemia Vera) It is a rare disorder, of unknown etiology (may be genetic) RBCs increase 6-12million/mm3 Peak incidence in the sixth decade Clinical Manifestations

o May be asymptomatico Patient angry face appearance.

Skin: purplish red (face, neck, ears, hands, feet. Superficial veins: dark, distended.

o headacheo dizzinesso tinnituso Spleenomegaly

Oral Manifestations:o Purplish red oral mucosa, gingiva & tongue.o Tongue as if painted crystal violeto Varicositis in the ventral tongueo Erythematous, edematous gingiva.o Petechiae, ecchymosis of oral mucosa.

Prognosis:o The patient blood is characterized by high viscositywhich increase the tendency for

thrombosis

o Transformation to acute myeloid leukemia. Treatment:

o Treatment of blood hyperviscosity by Phlebotomy (to remove RBCs) Chemotherapy Radioactive phosphorus (32P) Myelosuppressive therapy (Hydroxyurea, -interferon)

o low-dose aspirin therapy as prophylaxis of thrombotic complicationso Bone marrow transplantation

Oral considerations:o Complete blood count is essential before treatmento Reduce hemoglobin level below 16g/dl and hematocrit below 2%o Control of hemorrhage after dental surgery should be considered.


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Anemia

Etiology

Blood Loss

Iron Deficiencyanemia

Plummer VinsonSyndrome

DecreasedProduction

Perniciousanemia

Folic acid def.

Aplastic anemia

IncreasedDestruction

IntracorpuscularDefect

Extracorpuscular

Defect

Enviromentalfactor

Size

Microcytic Normocytic Macrocytic

Conc. of Hb

Hypochromic Normochromic

B) Anemia Decrease in the normal amount of the circulating RBCs & reduction in Hemoglobin (Hb)

concentration

1)Blood lossa. Iron Deficiency AnemiaMicrocytic, Hypochromic

It is a microcytic hypochromic anemia due to inadequate supply of iron for normalhemoglobin synthesis in developing erythroid cells in the bone marrow.

Cause:1. Chronic blood loss

from GIT: from hemorrhoids, peptic ulcers, esophageal varices, or carcinoma from menstrual cycle and uterine bleed2. Impaired iron absorption (gastrectomy, metabolic syndrome).

3. Inadequate iron intake.4. Increased iron requirement (e.g. pregnancy; added iron demands of the fetus)


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Clinical Features:1. General:

o Pallor (of skin & palpebral conjunctiva), Fatigue, dyspnea,palpitation, tachycardia.

2. Cutaneous:o Nails: pale, brittle, split, spoon shaped (Koilonychia).3. Oral Manifestations:o Glossitis with different degrees of atrophy of fungiform and filliform papillaeo Erythernatous mucositiso Recurrent aphthous stomatitiso Pale oral mucosao Angular cheilitiso Oral candidiasiso Burning mouth for several months to 1 years duration.

Lab. Findings:o RBCs (3 - 4 million/mm3).o Hb conc.o MCV decreasedo MCHo MCHCo Serum Fe.o TIBC increased Increase

Treatment:o Eliminate cause.o Ferrous sulfate administered at 325 mg (60 mg iron) orally three times dailyo Parental iron (in cases of iron malabsorption or partial gastrectomy).o High protein diet.

Dental considerations:o Physician consultation prior to surgical treatment is recommended.o When the hemoglobin is less than 8 g/dl, general anesthesia should be avoided and the

potential for clinical bleeding and faulty wound healing should he recognized.

o Narcotic use should be avoided for those with severe anemia,o Dentists should be aware that anemia places a patient at increased risk for ischemic

heart disease.

Decrease


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b.Plummer-Vinson Syndrome(Patterson-Kelly Syndrome)

(Sideropenic dyspliagia)

Microcytic, Hypochromic.

It is a rare syndrome with the classic triad1. Anemia

Pallor (of skin & palpebral conjunctiva), Fatigue, dyspnea, palpitation,tachycardia

Nails: pale, brittle, split, spoon shaped (Koilonychia).2. Glossitis

Smooth, Red, Painful atrophic tongue It is associated by angular cheilitis

3. Dysphagia

Patient complains of food sticking in the throat The dysphagia may be intermittent or progressive over years, is usually

painless and limited to solids, and sometimes is associated with weight loss.

It results from esophageal muscular degeneration and stenosis or webs ofthe esophageal mucosa

Relative degrees of achlorhydria are usually present Complications:

o Oral, pharyngeal or esophageal carcinoma. Mechanism:

o Mucosal atrophy due to depletion of iron. Treatment:

o Ferrous sulphate 200mg 3 times /day.o Glossitis improves within 3 weeks.o Angular cheilitis improves at slower rate.o In cases of significant obstruction of the esophageal lumen by esophageal webs/

strictures with persistent dysphagia mechanical dilation of the web may be required.

o patient should be followed closely since there is an increased risk of squamouscarcinoma of the pharynx and esophagus

2)Decreased RBCs ProductionMegaloblastic AnemiasMacrocytic Normochromic.

A group of disorder chiefly affects cells of rapid turnover; (hematopoitic cells, epithelial cells,GIT mucosa, and oral mucosa]

Deficiency of vit B 12 and folic acid deficiency are the major causes of Megaloblastic anemia.


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a.Pernicious Anemia Megaloblastic or pernicious anemia is an autoimmune disease resulting from autoantibodies

directed against intrinsic factor (a substance needed to absorb vitamin B12 from the

gastrointestinal tract) and gastric parietal cells.

Vitamin B12 is necessary for the formation of RBCs. Clinical Features:

o General:Pallor (of skin & palpebral conjunctiva), Fatigue, dyspnea, palpitation, tachycardiaNails: pale, brittle, split, spoon shaped (Koilonychia).

o Neurologic: tingling sensation fingers, toes numbness, incoordination.o Psychiatric ;Irritability, personality changes, Mild memory impairment, dementia,

Depression

o Cardiac: Possible increased risk of myocardial infarction and stroke Oral manifestations:

o Tongue: sore, fiery red, burning sensation in the tongue, lips, buccal mucosa, and othermucosal sites.

o The tongue and mucosa may be smooth or patchy areas of erythema.o dysphagia and taste disturbance may be presento Aphthous like ulcers

Laboratory Findingso RBCso Plateletso Increased MCVo MCHC Normal.o Megaloblastic marrow changeso Serum B 12 decreased (N. 200-600 pg/mi).o Circulating antibodies against Intrinsic factor.o Schilling test +veo Achlorhydria.

Treatment:o weekly intra- muscular injections of vitamin B12 or large oral doseso It should be continued for the rest of the patients lifeo It corrects the hematologic changes but only arrest not correct the neurologic changes.

b.Folic acid deficiency anemia Cause:

o Inadequate intake (diet devoid of leafy vegetables).o Increased demand in pregnancyo Intestinal malabsorption.o Drugs: barbiturates, phenytoin, oral contraceptives.

Clinical Features:o As pernicious anemia. No neurologic symptoms

Abnormally large.


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Oral manifestations:o Severe angular cheilitis.o Glossitis.o Recurrent aphthaeo Severe ulcerative stomatitis and pharyngitis

Lab. Findings:o MCV increasedo MCHCo Serum B12o Schilling test -veo Serum folate decreasedo (Folic acid N. value: S-20 g/ml).

Treatment: Oral folic acid tablets 1- 5mg/day

c. Aplastic AnemiaPancytopenia (bone marrow aplasia)

Normocytic normochromic

Aplastic anemia (AA) is a rare blood dyscrasia in which peripheral blood pancytopenia resultsfrom reduced or absent blood cell production in the bone.

Cause:o Idiopathic.o 2ry Bone Marrow toxicity Due to:

Viruses Radiation Toxins Drugs (as sulfonamides, anti-rheumatic & chloramphenicol). Chemicals as paint solvents, benzol.

Clinical Features:o Anemia: General manifestations of anemia.o Leukopenia: Increased susceptibility to infection. (Candidiasis and viral infection,)o Thrombocytopenia: Purpura and bleeding, epistaxis or gingival bleeding.

Oral manifestations:o Pallor, gingival bleeding, Petechiae & ecchymosis.o Ulcers as in neutropenia, surrounded by little erythema.o Advanced cases resemble acute leukemia.

Normal


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Lab. Findings:o MCVo MCHCo Hbo RBCso Plateletso WBCs

Treatment:o Eliminate cause.o Supportive therapy with blood transfusions to correct anemia and thrombocytopeniao immunosuppression (with antithymocyte globulins and cyclosporine) is effective at

restoring blood cell production in the majority of patient

3)Increased destruction of RBCs(Hemolytic Anemia)

The normal RBC life span is 90 to 120 days in the circulation. Hemolytic diseases result inanemia if the bone marrow is not able to replenish adequately the prematurely destroyedRBCs.

Accelerated destruction of RBCs can be caused by:1. Intra corpuscular defecto a molecular defect inside the red cello two types

Hemoglobinopathies ex) sickle cell anemia and thalassemia Enzymopathy: ex) glucose -6- phosphatase dehydrogenase deficiency

2. extra corpuscular defecto an abnormality in membrane structure and function

3. Environmental factoro Such as an autoimmune hemolytic disease, infection, hyperspleenism.

Normal

Decrease

Fanconi anemiao Fanconi anemia is inherited aplastic anemia that manifest in children

o Fanconi anemia is associated with brown skin pigmentation

o hypoplasia of kidney and spleen

o mental and sexual retardation


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Sickle cell anemia (HbSS)o Occurs mainly in blacks.o 2 chains are abnormal (Homozygous).o 75% Hb is HbS remainder is HbF.o Appears by 3 months of life.

Clinical manifestation:o This disease characterized by periods of latency interrupted by periods of acute crisis.o The patient shows marked under developmento Manifestations of anemia and hemolysis (pallor, fatigue, dyspnea and jaundice, Cardiac

failure may develop)

o Manifestation following stasis of blood and vasoocclusion (splenic infarction, leg ulcers,bone pain, stroke)

o Aplastic crisis may develop from infection or hypersensitivity reaction which results in bonemarrow suppression

Oral manifestations:o Oral mucosa pale, yellow tinge.o Delayed teeth eruption.o Enamel hypoplasia.o Increased overbite and overjet (maxillary overgrowth)o Susceptible osteomyelitis.o vasoocclusion and interruption of the blood supply can result in

anesthesia of the inferior alveolar nerve pulpal necrosis facial pain or sensory changes in the distribution of the inferior alveolar nerve

Radiographic changes:o It affect Jaw bones, skull, vertebrae, long bones:

a) Osteoporosis:

2ry to bone marrow hyperplasia to compensate short life span of RBCs.b) Osteosclerosis:

2ry to thrombosis acts nidus for calcification.o Lateral skull view:

Marked thickening of the diploe with trabeculae running perpendicular to outer andinner table and thin cortex give the characteristic (hair on end) pattern.

o Jaw bones: osteoporosis & Osteosclerosis.o Alveolar bone:

Trabecular pattern horizontal rows between roots --> step ladder appearance. Lamina dura: dense, distinct, intact,

Lab Findings:o Hb -->decreasedo Normocytic, Normochromic.o Sickling test +ve.o Hb electrophoresis.


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Treatment:o Only symptomatic treatmento The precipitating factors for sickling should be avoided.o Acute attack require (IV fluid, oxygen, antibiotics and analgesics and anti-inflammatory to

manage acute pain of vasoocclusive attacks)

o use of folic acid dietary supplemento Blood transfusion indicated during aplastic crisis

Dental Considerations:o Avoid dental procedures during crisis.o Local anesthesia recommended no vasoconstrictor.o General anesthesia is hazardous.o Infection treated immediately.o Surgery carried out under antibiotic cover.o Poor wound healingo Management of pain that may be due to: osteomyelitis, infarction, odontalgia

Thalassemia

Microcytic hypochromic Thalassemia is a genetic disorder of hemoglobin synthesis characterized by a disturbance of

either alpha or beta hemoglobin chain production.

As a result RBCs are microcytic hypochromic. It is classified into

o Alpha Thalassemia.o Beta Thalassemia

Thalassemia minor (trait) thalassemia major (coolys anemia)

Thalassemia minor (trait) () Heterozygous, usually asymptomatic. Aggravated by: pregnancy, illness. Resembles iron deficiency anemia. No therapy

Thalassemia major (Homozygous) ()Cooleys anemia (Mediterranean)

Clinical Features:1- Severe Anemia:

o Ashen gray skin color (due to combination of pallor, jaundice and hemosiderosis)o irritability, weakness, dyspnea,o sore painful tongue (decreased folate)o patient may present with Hepatospleenomegaly (due to extramedullary hemopoiesis)

2- Iron overload (Hemosiderosis):o Patientssurvival depends on repeated blood transfusion which results in iron overload.o Iron deposits causes functional abnormalities in various organs and tissues: heart, liver,

pancreas, skin.

o Parotid gland - painful swelling.


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3- Dentofacial changes:o Large head, mongoloid features frontal bossingo (prominent bone short nose, depressed nasal bridge)oMaxilla - enlarged maxillary overbiteoMandibular prominenceoForward drifting and spacing of teeth

Radiographic changes:A. Lateral skull view:

o Thickening of the diploe, Hairon end appearanceB. Alveolar bone:

o Decreased density, loss trabecular detail and thinning of thecortical bone.

o Rarefactions between roots of teeth which will lead to Chickenwire appearance.

Oral manifestations:oMarked development of maxilla > B.M expansion.oOpen bite, spacing & drifting of teeth.oOral mucosa pale (esp,, Hard & soft palate).oSore painful tongue (decreased folate).oPainful swelling of parotid gland-iron deposits.oDelayed dental developmentoShort rootsoDiscoloration of enamel and dentine due to iron deposition.

Diagnosis & Lab. Findings:oClinical appearance (Mongoloid face).oBlood film, (microcytic, hypochromic).oDecreased Hb and increased serum iron.oDecreased serum folate due to utilization for erythropoiesis.oHb electrophoresis: 90% HbF & rest HbA2

TreatmentoRegular blood transfusionoiron chelation by continuous injection of(deferoxamine) to resolve the iron overload,oTreatment of complications and possible infections

Glucose -6- phosphatase dehydrogenase deficiency it is an hereditary enzyme defect that causes episodic hemolysis because of a reduced

capacity of RBCs to deal with oxidative stresses.

patients are more likely to manifest neonatal jaundice risk of developing acute hemolytic anemia in response to: fava beans, viral or bacterial

infections, and drugs.


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White Blood Cell Disorder

A)Quantitative Leukocyte Disorder1. Granulocytosis

Increase in number of WBCs Neutrophilia (an excess of neutrophils) is more common. Causes of Neutrophilia are varied and include acute infections, acute myocardial infarction,

rheumatic fever, and hypersensitivity reactions, neoplastic diseases.

2. Agranulocytosis (granulocytopenia/Neutropenia) Mostly due to decrease in neutrophils (no neutrophils found in peripheral circulation). Etiology:

o Idiopathic.o Secondary: drugs, viral infection, antibody-mediated destruction, myelosuppression &

irradiation.

Clinical Features:o Fever, chills, fatigue.o Mucosal ulcers, acute pharyngitis.o Infection (characteristic feature) in the mouth, GIT, urinary tract, skin.o Regional lymphadenopathy

Oral Features:o Painful necrotizing ulcers: Gingiva, tongue & palate.o Ulcers: large, deep, irregular, necrotic, no inflammatory reaction at margins.o 2ry fusospirochetal infection foul odor.

Treatment:o Eliminate the cause.o Dental Management:

Parenteral antibiotics. Topical antibiotics Chlorhexidine mouth wash. Topical anesthesia for painful ulcers. No dental surgery performed.

3. Cyclic neutropenia Cyclic neutropenia is a rare hematologic disorder, characterized by repetitive episodes of

fever, mouth ulcers, and infections attributable to recurrent severe neutropenia.

Neutropenia recurs with a regular periodicity of 21 days, persists for 3 to 5 days. Infectious events are usually less severe than in severe chronic Neutropenia. It is an aitosom3-cIominant disease caused by a gene mutation It can during the first few years of life.


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Clinical and Oral Manifestationso Periodic oscillations of neutrophil counts associated with fever and mouth ulcers,

lymphadenopathy, and infections.

o Patient is healthy between neutropenic episodes Dental considerations:

o Most common 2 manifestation=oral mucosal ulcers and periodontal diseaseo Early management of infections and maintained good oral hygiene.o Recall patient every 2-4 months

B)Qualitative disorder1.Leukemia

Neoplastic proliferations of WBC, Abnormal increase in number of circulating immatureWBCs.

The malignant cells replace and turn off the normal marrow elements causing anemia,thrombocytopenia, and deficiency of normal functioning leukocytes.

Etiology:o Genetic factors play a role.o Individuals with down syndrome have increased incidence with leukemia.o Exposure to radiation with high dose, certain chemicals and drugs.

Leukemia is classified into

Acute leukemia Acute leukemia occur at any age ALL is commonly found in children AML is more frequently in adults The signs and symptoms results from either bone marrow suppression or infiltration of

leukemic cells into other organs Gingival hyperplasia secondary to leukemia cell infiltration may be a first sign

Leukemia

Acute

LymphocyticLeukemia

(ALL)

Myoloid Leukemia

(AML)

Chronic

Lymphocytic

Leukemia

Mylocytic

Leukemia


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Clinical manifestations:o Anemia: fatigue, malaise, pallor,o Thrombocytopenia: mucosal bleeding, Petechiae.o Decrease leukocytic function: fever, local infections (infection of lung, urinary tract

skin...), Lymphadenopathyo Localized tumors consisting of leukemic cell infiltrate.o Chemotherapy-induced mucositis and infection, including herpes simplex ulcers and oral

candidiasis, are commonly observed complications of leukemia in the oral cavity

Oral Manifestations:o Pallor of oral mucosa,o Atrophy of tongue coatingo Oral & gingival bleeding Petechiae & ecchymosiso Gingival Enlargemento Oral ulceration -->Ulcers large, deep irregular, No

inflammatory haloo Ulceration due Chemotherapyo Infection(Viral, Bacterial, Fungal)o Foul odor due to Spirochetal infection.

Oral Health Considerationso Prechemotherapy dental assessment, maintenance of oral hygiene, and management of

periodontal infection to prevent oral and systemic complications during treatment.

o Symptomatic treatment for oral ulcers & candidiasis.o Analgesic pain.o Antifungal antiviral for oral lesionso Drainage of abscessed tootho No surgery except emergency patient hospitalizedo Spontaneous gingival bleeding remove gross local factors, place subgingivally under

pressure absorbable gelatin saturated + thrombin

Chronic leukemia Characterized by the presence of large number of well differentiated cells in the bone

marrow.

This distinguish chronic leukemia from acute leukemia in which immature cells predominateand the untreated clinical course leads to death in months

less pronounced marrow failure than acute leukemiaChronic mylocytic leukemia

Disorder resulting in myeloid marrow hyperplasia. less pronounced marrow failure than acute leukemia have an indolent course that usually

lasts several years

Clinical manifestationo No symptoms are noted for 3 to 5 years in which patients have no symptoms followed

by an accelerated phase and blast crisis, resembling acute leukemia [fever, weakness,

fatigue, anorexia, weight loss, Spleenomegaly; anemia, and infection)

o Oral presentations are not common, resemble acute leukemia but are less severe,


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Chronic lymphocytic leukemia More than 90%of cases involve the B lymphocytes and 5% of cases account for T-

lymphocytes

B lymphocytes do not carry out their normal immunologic function and do not differentiateinto normal immunologic producing plasma cells when exposed to antigen

Clinical manifestationso asymptomatic phase last for yearso can be detected by routine hematology before any or when patients present with [fever,

night sweats, weight loss, fatigue),

o bone marrow infiltration causes anemia and thrombocytopeniao Leukemic cell infiltrate effects of other tissues as skin mucosa, liver and spleen.o Oral manifestations at presentation of CII are infrequent less severe than acute

leukemia, and generally related to bleeding.

o The oral lesion incidence rate increases once chemotherapy is initiated for treatment.Multiple myeloma

It is a plasma cell neoplasm that is characterized by a bone marrow plasmacytosis. Clinical Manifestations

o Symptoms include fatigue, weakness, weight loss, bone pain, and recurrent infections.o This disease is characterized by a high capacity to induce focal osteolytic bone lesions,

diffuse osteopenia, and pathologic fractures.

o This result from increased osteoclast formation, osteoblast inhibition induced by mmcells.

Oral Manifestationso Patients can manifest soft tissue masses of the jaws.o Punched-out lesions in the skull from the focal proliferation of plasmacells inside the

bone marrow, ranging from asymptomatic osteolytic lesions to pathologic fracture.

Lymphoma Classification

o Hodgkins Diseaseo Non- Hodgkins Lymphoma

Hodgkins disease Malignant neoplasm of lymphoid tissue. Etiology unknown. Asymptomatic. Enlarged cervical LNs. Discrete rubbery not tender. Clinical Features:

o LNs enlarged causing pressure on organs or ducts.o Retroperitoneal LNs --> urinary obstruction.o Mediastinal LNs -->Dysphagia, chest pain, cough, and dyspnea.o

BM invasion, invasion of spleen, liver, lung & spinal cord.

o night sweats, fever, and weight loss


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o Hodgkins disease rarely presents as an extra nodal mass in the head and neck region. Oral Health Considerations

o Patients who receive radiation in fields involving the cervical nodes will invariably havetheir submandibular and sublingual salivary glands in the field and are at risk for

temporary, and occasionally permanent, xerostomia.

Non-Hodgkins Lymphoma Malignant neoplasms, arise from lymph nodes. Nodular or diffuse. Mainly B-cell origin. Poor prognosis. Clinical manifestations Slowly progressive, usually painless peripheral lymphadenopathy, Extranodal involvement of the GIT, skin, bone marrow, sinuses, thyroid, or central nervous

system.

Systemic constitutional symptoms such as fever, chills, night sweats, and weight loss occur Oral manifestations:

o Hodgkins disease rarely affects mouth.o It may cause soft tissue swelling in pharynx, palate, tongue, gingiva or lips

-THE END-

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