Myeloproliferative Neoplasms other than Chronic Myeloid Leukemia - A Five Year Single Center Experience

National Institute of Blood Disease and Bone Marrow Transplantation Karachi, Pakistan *Corresponding Author’s Email: [email protected] Tel.: +923009370577 A retrospective analysis of 5 years data of myeloproliferative neoplasm (MPN) other than chronic myeloid leukaemia (CML) was done. All the cases were diagnosed and classified according to World Health Organization (WHO) Classification 2008 and studied for demographic, clinical, laboratory & molecular characteristics along with treatment. Out of total 89 cases, we found 37% cases of Polycythemia Vera (PV), 31% each of Essential Thrombocythemia (ET) and Primary Myelofibrosis(PMF) respectively. Haemoglobin (Hb) was found raised in PV, Platelets in ET but lesser than normal values were seen in PMF. Jak2 V617F mutation was detected in 54%, 35% and 11% cases of PV, ET and PMF respectively. Splenomegaly was found in 27%, 28% and 78% in PV, ET and PMF respectively. Hydroxyurea was given in 76% of PV and 71% of ET patients along with aspirin. Venesection was done in 51% of PV patients. However, thalidomide, steroids, androgens and azathioprine were given for PMF. We found relatively younger median age at presentation as compared to previous data. On account of considerable overlap in signs & symptoms of these disorders, molecular analysis are essential in diagnosis in addition to bone marrow biopsy. Lower frequency of Jak2 V617F positivity in our cohort might be due to the use of NESTED PCR and financial constraints. The use of newer molecular markers in future like Calreticulin, JAK2 exon12 and MPL mutations will fill the diagnostic gap to considerable extent in cases where JAK2 v671f mutation is not detectable in MPN.