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Other LeukocyteOther Leukocyte

DisordersDisorders

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Hema-20Hema-20 33

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Hema-20Hema-20 44

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Hema-20Hema-20 55

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Hema-20Hema-20 66

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Hema-20Hema-20 88

Myeloproliferative DisordersMyeloproliferative Disorders

Acute FAB M0 to M7Acute FAB M0 to M7 Chronic distinguished byChronic distinguished by

predominate cell typepredominate cell type Affects middle age & olderAffects middle age & older Insidious onsetInsidious onset BM fibrosisBM fibrosis TransitionsTransitions Platelet abonormalitiesPlatelet abonormalities

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Hema-20Hema-20 99

Chronic MyelogenousChronic Myelogenous

LeukemiaLeukemia

CML with all its variationsCML with all its variations

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Hema-20Hema-20 1010

Polycythemia veraPolycythemia vera

ErythrocytosisErythrocytosis

Primary causePrimary cause

Secondary causesSecondary causesNormal response to abnormal eventNormal response to abnormal event

AltitudeAltitude

Increases activityIncreases activityPregnancyPregnancy

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Hema-20Hema-20 1111

Polycythemia veraPolycythemia vera

Excessive proliferation ofExcessive proliferation of

myeloid & megakaryocyticmyeloid & megakaryocytic

elements in BM & Bloodelements in BM & Blood

Absolute increase in RBC massAbsolute increase in RBC mass

Abnormal pluripotent stem cellsAbnormal pluripotent stem cells

>50 yo, avg 60 yo>50 yo, avg 60 yo Ashkenazi Jews 10:1 ArabsAshkenazi Jews 10:1 Arabs

Etiology UnknownEtiology Unknown

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Hema-20Hema-20 1212

Polycythemia veraPolycythemia vera

Clinical featuresClinical featuresProgressive increase in Hct, Hgb,Progressive increase in Hct, Hgb,

RBCRBC

Headaches, dizziness, visionHeadaches, dizziness, vision

problemsproblems

Bleeding problemsBleeding problems

SplenomegalySplenomegalyRuddy ComplexionRuddy Complexion

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Hema-20Hema-20 1313

Polycythemia veraPolycythemia vera

Laboratory FindingsLaboratory Findings Increase RBC, Hct, HbgIncrease RBC, Hct, Hbg

May develop Iron deficiency,May develop Iron deficiency,

MicrocytosisMicrocytosis

Reticulocytes normalReticulocytes normal

Platelets increasePlatelets increase

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Hema-20Hema-20 1414

Polycythemia veraPolycythemia vera

TreatmentTreatmentPhlebotomyPhlebotomy

Tried P*, not goodTried P*, not good

Prognosis 8 15 yearsPrognosis 8 15 yearsButBut

BM wears ooutBM wears oout

Conversion to Acute formsConversion to Acute forms

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Hema-20Hema-20

1616

Essential ThrombocythemiaEssential Thrombocythemia

TreatmentTreatmentAspirinAspirin

ChemoChemo

InterferonInterferon

Prognosis > 5 yearsPrognosis > 5 years

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Hema-20Hema-20

1717

Myelofibrosis with MyeloidMyelofibrosis with Myeloid

MetaplasiaMetaplasia

Fibrosis of Bone MarrowFibrosis of Bone Marrow

Multiple possible symptomsMultiple possible symptoms

Age 50 70 yoAge 50 70 yo Male 2:1Male 2:1

Less common in AfricansLess common in Africans

Etiology unknownEtiology unknown

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Hema-20Hema-20 1818

MyelofibrosisMyelofibrosis

Decrease RBCDecrease RBC Increase PLTIncrease PLT Fibrosis in BM, Dry tapFibrosis in BM, Dry tap Splenomegaly, LiverSplenomegaly, Liver Anemia becomes progressiveAnemia becomes progressive Bizarre RBC morphologyBizarre RBC morphology Treat symptomsTreat symptoms Prognosis varies months to > 10yPrognosis varies months to > 10y

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Hema-20Hema-20 1919

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Hema-20Hema-20 2020

Myelodysplastic syndromesMyelodysplastic syndromes

Refractory Anemia (RA)Refractory Anemia (RA)

RA with ringed sideroblastsRA with ringed sideroblasts

RA with excess blasts (RAEB)RA with excess blasts (RAEB) RAEB in transformationRAEB in transformation

Chronic MyelomonocyticChronic Myelomonocytic

LeukemiaLeukemia

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Hema-20Hema-20 2121

Myelodysplastic syndromesMyelodysplastic syndromes

Elderly, ~ 70 yoElderly, ~ 70 yo

Few youngerFew younger

Etiology unknown, butEtiology unknown, but Toxins maybe linkedToxins maybe linked

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Hema-20Hema-20 2222

Refractory Anemia (RA)Refractory Anemia (RA)

Difficult to Recognize &Difficult to Recognize &diagnosisdiagnosis

FAB categoriesFAB categories Based on Qualitative differencesBased on Qualitative differences RARA

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Hema-20Hema-20 2323

RA with ringed sideroblastsRA with ringed sideroblasts

RARA15% ringed sideroblasts

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Hema-20Hema-20 2424

RA with excess blasts (RAEB)RA with excess blasts (RAEB)

RARA

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Hema-20Hema-20 2525

RAEB in transformationRAEB in transformation

RARA>5% Blasts>5% Blasts

CytopeniaCytopenia

3 different cell lines3 different cell lines

BM 20 - 30% blastsBM 20 - 30% blasts

5% 20% Auer Rods5% 20% Auer Rods

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Hema-20Hema-20 2626

Chronic MyelomonocyticChronic Myelomonocytic

LeukemiaLeukemia

RARA20% Monocytic