9960/3 Myeloproliferative disease, NOS



Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: D47.1
           

Myeloproliferative neoplasms (MPN) is a generic term for clonal haematopoietic stem cell disorders characterized by proliferation of one or more of the myeloid lineages (granulocytic, erythroid, megakaryocytic and mast cell)1
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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MPNs are associated with hypercellularity of the bone marrow (BM) with mature granulocytes, red blood cells and/or platelets in the peripheral blood (PB). Splenomegaly and hepatomegaly are common. MPN may lead to marrow failure due to myelofibrosis, but also has the potential to progress to an acute blast phase. The finding of 10−19% blasts in the PB or BM generally signifies accelerated disease and 20% or more is sufficient for a diagnosis of blast phase
2
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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Adults (5th to 7th decade) are predominatly affected. An annual incidence rate of 6−10 cases/100,000 population has been reported
3
Click to access Pubmed
Johansson P (2006)
Epidemiology of the myeloproliferative disorders polycythemia vera and essential thrombocythemia.
Semin Thromb Hemost 32: 171-3



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The designation of a case as MPN, NOS should be used only for cases of MPN for which sufficient clinical, morphological or molecular genetic data can not be obtained that would allow an accurate classification into one of the appropriate MPN catgories, including the category of MPN, unclassifiable.

The following myeloproliferative neoplasms are distinguished:
Chronic myelogenous leukemia (CML), BCR-ABL positive
Chronic neutrophilic leukemia
Polycythemia vera (PV)
Primary myelofibrosis (PMF)
Essential thrombocythemia (ET)
Chronic eosinophilic leukemia, NOS
Myeloproliferative neoplasm, unclassifiable

Cutaneous mastocytosis
Systemic mastocytosis
Mast cell leukaemia
Mast cell sarcoma
Extracutaneous mastocytoma