Other Leukocyte Disorders

Hema-20

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Hema-20

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Hema-20

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Hema-20

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Hema-20

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Myeloproliferative Disorders

  

Chronic Myelogenous Leukemia Polycythemia vera Essential Thrombocythemia Myelofibrosis with Myeloid Metaplasia

Hema-20

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Myeloproliferative Disorders
      

Acute – FAB M0 to M7 Chronic distinguished by predominate cell type Affects middle age & older Insidious onset BM fibrosis Transitions Platelet abonormalities
Hema-20 8

Chronic Myelogenous Leukemia

CML with all its variations

Hema-20

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Polycythemia vera
  

Erythrocytosis Primary cause Secondary causes
 Normal  Altitude  Increases

response to abnormal event

activity  Pregnancy

Hema-20

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Polycythemia vera

    

Excessive proliferation of myeloid & megakaryocytic elements in BM & Blood Absolute increase in RBC mass Abnormal pluripotent stem cells >50 yo, avg 60 yo Ashkenazi Jews 10:1 Arabs Etiology Unknown
Hema-20 11

Polycythemia vera

Clinical features
 Progressive

increase in Hct, Hgb,

RBC  Headaches, dizziness, vision problems  Bleeding problems  Splenomegaly  Ruddy Complexion
Hema-20 12

Polycythemia vera

Laboratory Findings
 Increase

RBC, Hct, Hbg  May develop Iron deficiency, Microcytosis  Reticulocytes normal  Platelets increase

Hema-20

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Polycythemia vera

Treatment
 Phlebotomy  Tried

P*, not good

Prognosis 8 – 15 years
 But…  BM

wears oout  Conversion to Acute forms

Hema-20

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Essential Thrombocythemia

Increase in Platelets
>

600,000  May be > 1,000,000
  

Vascular blockage Decrease in PLT size, use PDW WBC increase

Hema-20

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Essential Thrombocythemia

Treatment
 Aspirin  Chemo  Interferon

Prognosis > 5 years

Hema-20

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Myelofibrosis with Myeloid Metaplasia
     

Fibrosis of Bone Marrow Multiple possible symptoms Age 50 – 70 yo Male 2:1 Less common in Africans Etiology unknown

Hema-20

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Myelofibrosis
       

Decrease RBC Increase PLT Fibrosis in BM, Dry tap Splenomegaly, Liver Anemia becomes progressive Bizarre RBC morphology Treat symptoms Prognosis varies months to > 10y
Hema-20 18

Hema-20

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Myelodysplastic syndromes
    

Refractory Anemia (RA) RA with ringed sideroblasts RA with excess blasts (RAEB) RAEB in transformation Chronic Myelomonocytic Leukemia

Hema-20

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Myelodysplastic syndromes
   

Elderly, ~ 70 yo Few younger Etiology unknown, but Toxins maybe linked

Hema-20

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Refractory Anemia (RA)
   

Difficult to Recognize & diagnosis FAB categories Based on Qualitative differences RA
 <1%

Blasts  Cytopenia  1-3 different cell lines  <15% ringed sideroblasts
Hema-20 22

RA with ringed sideroblasts

RA
 <1%

Blasts  Cytopenia  1-3 different cell lines  >15% ringed sideroblasts

Hema-20

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RA with excess blasts (RAEB)

RA
 <5%

Blasts  Cytopenia  2-3 different cell lines  variable ringed sideroblasts

Hema-20

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RAEB in transformation

RA
 >5%

Blasts  Cytopenia  3 different cell lines  BM 20 - 30% blasts  5% 20% Auer Rods

Hema-20

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Chronic Myelomonocytic Leukemia

RA
 <5%

Blasts  Monocytosis  2-3 different cell lines  >20% Monocytic

Hema-20

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