Clinical Characteristics and Prognostic Risks of Philadelphia-Negative Myeloproliferative Neoplasms at Cipto Mangunkusumo General Hospital

BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPNs) are clonal hematopoietic cell malignancy that comprises polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). They prone to develop thrombosis, bleeding, fibrotic progression, and leukemic transformation. We aimed to study clinical characteristics, thrombosis complications, and prognostic risk in Indonesians with Ph-MPNs. METHODS: This was a single-center retrospective cross-sectional study, including patients with Ph-MPNs who attended Hematology and Medical Oncology outpatient clinic at Cipto Mangunkusumo General Hospital, Jakarta, between 2016 and 2021. Demographic data, clinical characteristics, previous antiplatelet treatment, baseline laboratory data, JAK2V617F mutation, and treatment were reviewed for analysis. The prognostic risk model was assessed for PV (IPSS), ET (IPSET-thrombosis), and PMF (IPSS and DIPPS) patients. RESULTS: One hundred forty-six patients were classified as PV (31.5%), ET (38.4%), and PMF (30.1%) with median ages of 54, 53.5, and 55 years, respectively. PMF presented with the most diverse clinical presentations. JAK2V617 mutations were detected in 82%, 42.5%, and 76.5% of PV, ET, and PMF patients. PV had the highest thrombosis event pre/at diagnosis (26.1%), with predominantly arterial thrombosis. Bleeding occurred more in PMF (20.5%). Phlebotomy and hydroxyurea are the only treatment available for Ph-MPNs in the government hospital in Indonesia. IPSS intermediate risk in PV (47.8%), IPSET-thrombosis low risk in ET (46.4%), and IPSS and DIPSS intermediate-2 in PMF (38.6% and 50% respectively) were the most common risk groups. CONCLUSION: Essential thrombocythemia was the most prevalent Ph-MPNs in Indonesia. Arterial thrombosis was the highest thrombosis event pre/at diagnosis in PV despite IPSET-thrombosis low risk being the most common risk group. In JAK2V617 mutation-positive MPN population, thrombosis event was also the highest in PV.