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Background: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by abnormal proliferation of megakaryocytes, bone marrow fibrosis, and extramedullary hematopoiesis. We did mutation profile of 50 patients of PMF and tried to correlate it with initial clinical presentation of these patients. Materials and Methods: All new and follow up patients who were diagnosed as PMF based on WHO 2016 definition of PMF were included. Mutation profile of these patients including JAK2 V617F, JAK2 exon 12, CALR and MPL mutations was done and all clinical, demographic and laboratory details were recorded. Results: Total 50 patients were enrolled out of which 29 were males and 21 were females. Out of these patients, 32 (64%) were JAK2 positive, 13 (26%) were CALR positive, 1 (2%) were MPL positive and 4 (8%) were triple negative. As compared to JAK2+ve patients and triple negative group, CALR positive patients were younger, had lower total leucocyte count, larger spleen size, lower dynamic international prognostic scoring system (DIPSS) score and higher grade of fibrosis of marrow. Conclusion: This study depicts that incidence of JAK2 and CALR mutations in Indian PMF patients is fairly similar to that in rest of the world. CALR positive patients have better clinical parameters at presentation and have better prognosis as compared to JAK2 positive patients.

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Clinical ProblemA 3-years-old male child was hospitalized for rightsided pneumonia. Complete blood count (CBC) showed hemoglobin 13.5 gm/dl, total leucocyte count-6800 cells/cumm and thrombocytopenia with a platelet count of <20,000 cells/cumm on two occasions. There was no history of easy bruisability, petechiae, ecchymosis or bleeding from any site. There was no history of bleeding disorders in family members. Physical examination was normal. CBC by an automated counter in the hematology lab showed hemoglobin of 13 gm%, total leucocyte counts of 10,500 cells/cumm and platelet counts of 10,000 cells/cumm. A peripheral smear examination showed multiple aggregates of platelets (Figure 1). All previous CBCs were done using ethylenediaminetetraacetic acid (EDTA) as an anticoagulant. When the CBC was repeated with sodium citrate as an anticoagulant, the platelet count was found to be 2,35,000 cells/cumm.

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