Characteristics of Patients With Philadelphia Negative Myeloproliferative Neoplasms at Assiut University Hospital

Diagnosis of MPN according to the revised 2008 WHO criteria is based on a. combination of clinical, morphological, and molecular parameters ( Cardinal features of Philadelphia chromosome-negative MPN are increased red blood cell (RBC). production in PV, sustained thrombocytosis in ET, and bone marrow (BM) fibrosis in PMF. Molecular markers, such as JAK2 V617F, JAK2 exon 12, and MPL mutations, have increased our knowledge of the pathogenesis of MPNs as well as the accuracy of diagnosis. Furthermore, many recent studies have reported that the calreticulin mutation (mutation in exon 9 of CALR) is found in the majority of patients with MPN with non mutated JAK2 . There are many controversies with regard to the subjectivity and the lack of reproducibility of the histological criteria. And BM assessment is not always needed, especially in PV. However, histologic evaluation of the pathologic features of megakaryocytic, granulocytic, and erythroid series and cellularity is important in differentiating MPNs, especially ET and PMF . Sometimes, patients with occult MPN present with normal blood counts because of gastrointestinal bleeding and associated iron deficiency, splenomegaly, or simply because they are in an early stage of the disorder . In cases of clinically suspicious MPN, such as intra-abdominal thrombosis with normal blood counts, careful investigation is needed.

diagnostic criteria for myeloproliferative (World Health Organization) 2008. Polycythemia refers to an increase in the number of red blood cells in the body. The extra cell cause the blood to be thicker, and this, in turn, increases the risk of other health issues, such as blood clots.

Maior criteria including. Hemoglobin > 18.5 g/dL (men), > 16.5 g/dL (women) or Presence of JAK2 V617F or JAK2 exon 12 mutation Minor criteria: BM hypercellularity with trilineage myeloproliferation Subnormal serum erythropoitin. Endogenous erythroid colony formation in vitro.

Management of polycythemia: The most common treatment for polychythemia vera is having frequent blood withdrawals, using a needle in a vein (phlebotomy).

Drugs that reduce the number of red blood cells If phlebotomy alone doesn't help enough, your doctor may suggest medications that can reduce the number of red blood cells in your bloodstream. Examples include: Hydroxyruea (Droxia, Hydrea), Interferon alfa-2b (Intron A), Ruxolitinib (Jakafi), Busulfan (Busulfex, Myleran) .