Other Leukocyte Disorders

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Other Leukocyte Disorders
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Myeloproliferative Disorders

• Chronic Myelogenous Leukemia
• Polycythemia vera
• Essential Thrombocythemia
• Myelofibrosis with Myeloid Metaplasia

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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

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Chronic Myelogenous Leukemia

• CML with all its variations

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Polycythemia vera

• Erythrocytosis
• Primary cause
• Secondary causes
• Normal response to abnormal event
• Altitude
• Increases activity
• Pregnancy

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Polycythemia vera

• Excessive proliferation of myeloid
  megakaryocytic elements in BM Blood
• Absolute increase in RBC mass
• Abnormal pluripotent stem cells
• gt50 yo, avg 60 yo
• Ashkenazi Jews 101 Arabs
• Etiology Unknown

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Polycythemia vera

• Clinical features
• Progressive increase in Hct, Hgb, RBC
• Headaches, dizziness, vision problems
• Bleeding problems
• Splenomegaly
• Ruddy Complexion

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Polycythemia vera

• Laboratory Findings
• Increase RBC, Hct, Hbg
• May develop Iron deficiency, Microcytosis
• Reticulocytes normal
• Platelets increase

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Polycythemia vera

• Treatment
• Phlebotomy
• Tried P, not good
• Prognosis 8 15 years
• But
• BM wears oout
• Conversion to Acute forms

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Essential Thrombocythemia

• Increase in Platelets
• gt 600,000
• May be gt 1,000,000
• Vascular blockage
• Decrease in PLT size, use PDW
• WBC increase

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Essential Thrombocythemia

• Treatment
• Aspirin
• Chemo
• Interferon
• Prognosis gt 5 years

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Myelofibrosis with Myeloid Metaplasia

• Fibrosis of Bone Marrow
• Multiple possible symptoms
• Age 50 70 yo
• Male 21
• Less common in Africans
• Etiology unknown

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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 gt 10y

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Myelodysplastic syndromes

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

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Myelodysplastic syndromes

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

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Refractory Anemia (RA)

• Difficult to Recognize diagnosis
• FAB categories
• Based on Qualitative differences
• RA
• lt1 Blasts
• Cytopenia
• 1-3 different cell lines
• lt15 ringed sideroblasts

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RA with ringed sideroblasts

• RA
• lt1 Blasts
• Cytopenia
• 1-3 different cell lines
• gt15 ringed sideroblasts

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RA with excess blasts (RAEB)

• RA
• lt5 Blasts
• Cytopenia
• 2-3 different cell lines
• variable ringed sideroblasts

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RAEB in transformation

• RA
• gt5 Blasts
• Cytopenia
• 3 different cell lines
• BM 20 - 30 blasts
• 5 20 Auer Rods

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Chronic Myelomonocytic Leukemia

• RA
• lt5 Blasts
• Monocytosis
• 2-3 different cell lines
• gt20 Monocytic