Clinical Manifestations and Risk Factors for Complications of Philadelphia Chromosome-Negative Myeloproliferative Neoplasms

• DOI: 10.7314/apjcp.2015.16.12.5013
• Publication Date: 2015-09-10T14:37:29Z
• Authors: Boonlerd Duangnap...; Ekarat Rattaritta...; Thanawat Rattanat...; Sasinee Hantrakool; Chatree Chai-Adis...; Adisak Tantiworawit; Lalita Norasetthada
• Keywords: Myeloproliferative neoplasm; Polycythaemia; Philadelphia chromosome; Janus kinase 2; Hematology

Background: Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell disorders characterized by proliferation of one or more myeloid lineages. Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) classical Philadelphia chromosome (Ph)-negative MPN that have a Janus Kinase 2 (JAK2) mutation, especially JAK2V617F in the majority patients. The major complications Ph-negative MPNs thrombosis, hemorrhage, leukemic transformation. Objective: To study clinical manifestations including symptoms, signs, laboratory findings, mutations (PV, ET PMF) as well their complications. Materials Methods: All patients who attended Hematology Clinic at Maharaj Nakorn Chiang Mai Hospital from January, 1 2003 through December, 31 2013 were retrospectively reviewed for demographic data, characteristics, complete blood count, mutation analysis, treatment, Results: One hundred fifty seven included study. They classified PV, PMF 68, 83 6 with median ages 60, 61, 68 years, respectively. detected 88%, 69%, 100% PV had highest incidence thrombosis (PV 29%, 14%, 0%) occurred both arterial venous sites whereas bleeding (PMF 17%, 11%, 7%). During follow up, there was patient transformed to acute leukemia five cases developed (three two patients). No secondary death encountered. Conclusions: various manifestations. present ET, This confirmed most significant MPN.