Depika Sanasam1 , Banashree Devi Rajkumari2 , Anil Irom3 , Sushila Devi L. 4
BACKGROUND Myeloproliferative neoplasms (MPN) are a group of haematopoietic stem cell disorders leading to clonal myeloproliferation characterized by granulocytosis, erythrocytosis and / or thrombocytosis. Myelodysplastic / myeloproliferative neoplasms (MDS / MPN) are neoplasms of the haematopoietic stem cells which show features of both myeloproliferative and myelodysplastic neoplasms. Incidence of MPN and MDS / MPN are sparse due to lack of proper data or registry. We wanted to evaluate the spectrum of MPN and MDS / MPN cases diagnosed in a tertiary care hospital within a two and a half year period. METHODS This is a retrospective cross-sectional study. All the information was obtained from the bone marrow and clinical OPD registers maintained in the concerned departments. Correlation of complete haemogram & bone marrow findings with molecular and genetics analysis was also done in some of the cases. The 2017 revised edition of WHO classification of tumours of haematopoietic and lymphoid tissues was adopted for categorization. RESULTS A total of 42 cases was studied including 40 cases of MPN and 2 cases of MDS / MPN. Out of the 40 cases of MPN, 20 cases of chronic myeloid leukaemia (CML), 14 cases of polycythaemia vera (PV), 3 cases of primary myelofibrosis (PMF), and 3 cases of essential thrombocythaemia (ET) were observed. Amongst the CML cases, 30 % were in blast crisis at presentation. All the cases with lymphoid blast crisis were of B-cell phenotype. Molecular study for BCR-ABL1 was done for all the cases. JAK2V617F and CALR mutation was noted in 67 % of PMF and ET cases respectively. Monosomy 7 was detected in 1 case of Juvenile myelomonocytic leukaemia (JMML). CONCLUSIONS In our study, we found certain clinico haematological profiles different from those of other studies which may be contributed to geographical and racial distribution. However, a thorough health investigation is needed.
