[Analysis of Incidence Rate, Risk Factors and Prognosis of Pulmonary Hypertension in Ph-MPNs Patients].

Objective: To explore and analyze the incidence rate, influencing factors and impact on prognosis of pulmonary hypertension (PH) in patients with Philadelphia chromosome negative myeloproliferative neoplasms (Ph^- MPNs).

Methods: The clinical data of 271 patients with Ph^- MPNs were retrospectively analyzed, and different disease subtypes were classified. Patients with different disease types were further divided into PH⁺ and PH^- groups according to whether HP occurred. Statistical methods were used to analyze the incidence rate, risk factors, and impact on prognosis of PH in Ph^- MPNs patients.

Results: The overall incidence rate of PH among 271 patients was 26.9%, and according to the classification of disease subtypes, it was found that the incidence rate of PH in patients with primary myelofibrosis (PMF) was significantly higher than those of patients with polycythemia vera and essential thrombocythemia (both P <0.05). Multivariate regression analysis showed that advanced age, long disease course, JAK2 positive and increased hematocrit, lactate dehydrogenase, monocyte count, and uric acid level were independent risk factors for PH in Ph^- MPNs patients (OR >1, P <0.05), and there were some differences in the independent risk factors between different disease subtypes. Survival analysis results showed that the overall survival (OS) rate of PH⁺ patients was significantly lower than that of PH^- patients in other types except for PMF (all P <0.05).

Conclusion: The incidence rate of PH in Ph^- MPNs patients is high, and its risk factors are diverse. The OS rate of Ph^- MPNs patients with PH is low. Therefore, we should be highly alert to the occurrence of PH in Ph^- MPNs patients clinically.