Clinicopathological characteristics and outcome of patients with Philadelphia negative myeloproliferative neoplasms: first report from Upper Egypt

Background
Philadelphia chromosome-negative myeloproliferative neoplasms (Ph-ve MPNs) are clonal hematopoietic stem cell neoplasms, include polycythemia vera (PV), primary myelofibrosis (PMF), and essential thrombocythemia (ET). Patients more likely to develop thrombosis, fibrotic progression, and leukemic transformation.


Objectives
To evaluate clinico-pathological characteristics and outcomes of Ph-ve MPNs patients.


Patients and methods
This prospective study included 98 Ph-ve MPNs patients. Clinical characteristics, laboratory data, bone marrow cytogenetic, the Janus kinase 2 (JAK2) V617F mutation, and treatment were recorded. The prognostic risk model was estimated. The response criteria were evaluated. Patients were re-assessed after 3 months and followed-up for 2 years.


Results
Out of 98, 48 (49.1%) PV, 32 (32.7%) PMF, and 18 (18.4%) ET. ET patients were much younger than PV or PMF patients (P=0.042). PMF patients were more likely to experience fatigue, fever, and anemic manifestation than PV or ET patients (P<0.05), and had more splenic enlargement than PV or ET patients (P<0.001). Also, PMF patients had higher MPN scores than PV or ET patients (P<0.001). Survival rates were 95.8% in PV, 62.5% in PMF, and 100% in ET patients (P<0.001). Kaplan–Meier analysis revealed an 84.8% OS rate.


Conclusion
PV was the most prevalent ET has the highest thrombotic events. Ph-ve MPNs patients have a severe disease burden and poor outcomes.