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Abstract
Chronic Myeloproliferative Neoplasms (CMPNs) are clonal haematopoietic stem cell disorders characterized by proliferation of one or more myeloid cell lineages in the bone marrow and increased numbers of mature and immature cells in the peripheral blood. CMPNs include polycythaemia vera (PV), essential thrombocythaemia (ET), primary myelofibrosis (PMF) and chronic myeloid leukemia (CML) plus rarer subtypes such as chronic neutrophilic leukemia(CNL) and chronic eosinophilic leukemia(CEL). These disease overlap with myelodysplastic/ myeloproliferative neoplasms (MDS/MPN) such as atypical CML (aCML) and chronic myelomonocytic leukemia(CMML) in which proliferation is accompanied by dysplastic features or ineffective haemopoiesis.
The major subgroups of myeloid neoplasms accordin to the WHO includes Myeloproliferative neoplasm(MPN), myelodysplastic syndromes (MDS), myelodysplastic/myeloproliferative neoplasm (MDS/MPN), acute myeloid leukemia.
The myeloproliferative neoplasms include: chronic myeloid leukemia (CML) , chronic neutrophilic leukemia, polycythaemia vera (PV), essential thrombocythaemia (ET), primary myelofibrosis (PMF) , chronic eosinophilic leukemia (CEL NOS) , mastocytosis and myeloproliferative unclassifiable (MPN,U)
True polycythaemia refers to as absolute increase in total body red cell volume (or mass) which usually manifests itself as a raised haemoglobin (Hb) concentration and/ or packed cell volume (PCV). A raised Hb (or PCV) can also be secondary to a reduction in plasma volume, without an increase in total red cell volume which is known as apparent (or relative ) polycythaemia.
WHO diagnostic criteria of PV: diagnosis requires both major and one minor criterion or the presence of 1st major together with two minor criteria.