Assessment of Clinical Manifestation and Prognostic Risks for Patients with Philadelphia –Negative Myeloproliferative Neosplasms (MPN) by IPSET- Thrombosis, IPSS and DIPPSS Model

Abstract

Objective: To evaluate clinical patterns, prognostic risks, as well as thrombotic complications in patients with Philadelphia – negative myeloproliferative neoplasms.

Methodology: This prospective, cross-sectional study was carried out at Department of Pathology, Chandka Medical College, SMBBMU Larkana between March 2021 to October 2022. Demographic variables, clinical patterns, previous history of treatment, current baseline data of laboratory, mutation status, and management history were analyzed. Assessment of prognostic risk model was done as: ET (IPSET-thrombosis), PV (IPSS), and PMF (IPSS and DIPPS).

Results: Total of 73 participants were chosen for the study and were categorized into polycythemia vera (31.5%), essential thrombocythemia (38.35%), and primary myelofibrosis (30.13%) with mean ages of 52.23, 54.04, and 53.51 years, respectively. The most diverse clinical manifestations were observed in primary myelofibrosis. Mutation in JAK2V617F was detected in PV (78.26%), ET (42.85%), and PMF (77.27%). The highest events of thrombosis were seen in PV (26.08%), with predominant arterial thrombosis. The common risk groups were ISPSS intermediate-risk in patients with PV, IPSET-thrombosis low-risk in patients with ET, and IPSS and DIPSS intermediate-2 among patients with primary myelofibrosis.

Conclusion: Among all patients of Philadelphia – negative myeloproliferative neoplasms, essential thrombocythemia (ET) had the most prevalent rate. The highest thrombotic event was arterial thrombosis in polycythemia vera (PV) in spite of common group of low-risk IPSET-thrombosis. In patients with positive JAK2V617 mutation, thrombotic events were also high in polycythemia vera (PV).