Clinical Profile of Subjects with Myeloproliferative Neoplasms other Than Chronic Myeloid Leukemia
Journal Title: International Journal of Contemporary Medical Research - Year 2017, Vol 4, Issue 2
Abstract
Introduction: Myeloproliferative neoplasms (MPN) are rare heterogeneous group of disorders characterized by increased proliferation of the erythroid, megakaryocytic, and myeloid lineages. The Janus-associated Kinase-2 mutation JAK2 V617F in MPNs has been described as a frequent genetic event in majority of patients with polycythemia vera (PV), essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF). Study aimed to clinical and laboratory evaluation of subjects with myeloproliferative neoplasms other than CML, as diagnosed by 2008 WHO criteria Materials and Methods: All patients presenting to outpatient department and admitted as inpatient, with clinical features suggestive of MPN were evaluated (after thorough history and physical examination) with complete hemogram, biochemical investigations, bone marrow aspiration, biopsy and cytogenetics. Analysis for JAK2 V617F mutation was performed. Diagnosis was made using revised WHO 2008 diagnostic criteria. Results: A total of 36 patients were evaluated. IMF was more common than PV and ET in our study. IMF patients were older compared to PV and ET. PV patients common symptoms were headache, flushing, erythromelalgia. Symptomatic anemia and splenomegaly were common in IMF. Thrombosis and bleeding were common in ET. JAK2 V617F mutation was detected in 75% of patients with PV, 71.4% in ET and 33.3% in IMF. JAK2 V617F detection was associated with older age of presentation, greater risk of thrombosis and higher values of hemoglobin, total leukocyte and platelet counts. Only one patient with PV developed Acute Myeloid Leukemia after one year of diagnosis. Conclusion: The 2008 WHO classification of myeloproliferative neoplasms is useful in diagnosing these patients and as the emphasis is on molecular abnormalities such as JAK2 V617F, it is easy to exclude secondary causes. The JAK2 V617F mutation also serves as a good prognostic marker indicating more severe forms of disease in positive patients. Regular follow up is essential to detect occurrence of other neoplasms like acute myeloid leukemia and evolution into other forms of MPNs.
Authors and Affiliations
Mallikarjuna Shetty, Anukonda Moti Venkata Raja Narendra, Nageswar Rao Modugu
Keywords
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