Chronic

Myeloproliferative
Disorders (CMPDs)
 CMPDs
AGENDA
Definition
Pathogenesis
Epidemiology
Clinical Features
Classification
CMPDs
Definition
- Clonal hematopoeitic stem cell disorders.
- Proliferation of one or more of the myeloid
lineage.
- Effective normal maturation increased
numbers of granulocytes, RBCs and/or
platelets.
 CMPDs
AGENDA
 Definition
 Pathogenesis
 Epidemiology
 Clinical Features
 Classification
 CMPDs
Pathogenesis
- Genetic abnormality occurs in hematopoietic
stem cells.
- No specific genetic abnormalities have been
identified in most CMPDs.
- Abnormal activation of tyrosine kinase signal
transduction
pathways.
- Increased transcription of genes that block apoptosis
 CMPDs
AGENDA
 Definition
 Pathogenesis
 Epidemiology
 Clinical Features
 Classification
CMPDs
Epidemiology
- Disease of adult (5th-7th decade).
- 6-9/100,000 population annually.
- Asia < Europe and America.
 CMPDs
AGENDA
 Definition
 Pathogenesis
 Epidemiology
 Clinical Features
 Classification
CMPDs
Clinical Features
• Insidious clinical onset
• Splenomegaly and/or hepatomegaly due to:
- sequestration
- extramedullary hematopoiesis
- leukemic infiltration
- combination
• Potential to progress to bone marrow failure
due to myelofibrosis or transformation to
acute leukemia.
• Overlap is frequent
 CMPDs
AGENDA
 Definition
 Pathogenesis
 Epidemiology
 Clinical Features
 Classification
CMPDs
Classification
• Based on:
- Lineage of predominant proliferating cells
- Prominence o bone marrow fibrosis
- Clinical features
- Laboratory features including:
- PBS examination
- BM examination
- Cytogenetics
- Molecular technique data.
 CMPDs
 Classification (cont’d)
• CML
• Polycythemia Vera (PV)
• Chronic Idiopathic Myelofibrosis (CIMF)
• Essential Thrombocythemia (ET)
 CMPDs
• Polycythemia Vera (PV)
 Definition
- Increased RBC production (autonomous).
- +/- excessive myeloid and megakaryocytic
proliferation .
 Clinical Features
- Related to increased RBC mass
- Venous or arterial thrombosis
- Headache, dizziness or visual disturbances.
- Pruritis
- Physical Examination: plethora, +/-splenomegaly,
+/- hepatomegaly
 CMPDs
• PV (cont’d)
 Diagnosis
- WHO criteria
- CBC :
elevated Hgb, Hct
+/-Neutrophils
+/- basophils
+/- thrombocytosis

- Morphology: PBS examination

BM examination
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• PV (cont’d)
 Complications
- Myelofibrosis
- Transformation into acute leukemia (~ 5-
10%)
 CMPDs
• CIMF
Definition
- Proliferation of mainly megakaryocytic and
myeloid
elements in the bone marrow with reactive
bone
marrow fibrosis and extramedullary
hematopoiesis.
Clinical Features
- 30% are asymptomatic (incidental findings).
- Fatigue, weight loss, night sweats, fever,
CMPDs
• CIMF (cont’d)
Diagnosis
CBC:
- variable (Prefibrotic or Fibrotic stage)
- +/- anemia
- +/- leukocytosis, Leukopenia or normal
WBCs
- +/- thrombocytosis, thrombocytopenia,
normal

Morphology: - PBS examination:

Leukoerythroblastic picture
Tear drop RBCs & poikilocytosis
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• CIMF (cont’d)
 Complications
- Bone marrow failure.
- Acute leukemia (5-30 %).
 CMPDs
• ET
 Definition
- Proliferation of megakaryocytic lineage in the
BM.
- Sustained thrombocytosis in the blood.
 Clinical Features
- Asymptomatic (50%)
- Vascular occlusions (arteries or veins)
- Bleeding (platelet dysfunction)
- +/- Hepatosplenomegaly
 CMPDs
• ET (cont’d)
Diagnosis
WHO criteria: - sustained thrombocytosis >600 x
109/L
- Megakaryocytic hyperplasia in BM
- Exclusion of other CMPDs
- Exclusion of secondary causes
CBC: - Thrombocytosis
Morphology : - PBS examination
- BM examination (Megakaryocytic
hyperplasia)
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• ET (cont’d)
Complications
- Myelofibrosis.
- Transformation into acute leukemia (~
5%).
CMPDs

OVERLAP in
Clinical & Laboratory Features

CMPD (NOS)