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chronic myeloproliferative disorder
Clonal hematopoietic stem cell disorders characterised by proliferation in the bone marrow of one or more myeloid lineage cell; associated with relatively normal maturation and increased numbers in peripheral blood. Differs from the ineffective hematopoiesis seen in myelodysplastic syndromes.
Classification: WHO
- chronic myelogenous leukemia
- chronic neutrophilic leukemia
- chronic eosinophilic leukemia and hypereosinophilic syndrome
- polycythemia vera
- chronic idiopathic myelofibrosis (with extramedullary hematopoiesis)
- essential thrombocythemia
- chronic myeloproliferative disease, unclassified
Etiology:
- no specific genetic abnormalities identified
- activation of tyrosine kinase signal transduction pathways frequently implicated
Epidemiology:
- primarily disease of adults, peak 5th - 7th decades
- 6-9/100,000 annually
Genetics:
- chromosomal aberration involving ETV6 is a cause of chronic myeloproliferative disorder with eosinophilia
Clinical manifestations:
- insidious onset
- splenomegaly, hepatomegaly common
- sequestration of excess cells
- extramedullary hematopoiesis
- leukemic infiltration
Disease progression:
- potential to undergo clonal evolution
- bone marrow failure
- myelofibrosis
- ineffective hematopoiesis
- transformation to acute blast phase
Laboratory:
- complete blood count
- bone marrow biopsy
- 10-19% blasts in blood or bone marrow may signify disease acceleration
- 20% or more blasts suffieient for diagnosis of blast phase
Specific
chronic myeloid leukemia (CML, granulocytic leukemia)
chronic myelomonocytic leukemia (CMML)
polycythemia rubra vera (PRV, PV, erythremia)
General
chronic hematologic disease (chronic blood disorder)
myeloproliferative disorder
Database Correlations
OMIM 131440
References
- WHO Classification of Tumours. Tumors of Haematopoietic and
Lymphoid Tissues. Jaffe et al. IARC Press 2001