Transformed to myelofibrosis is a risk factor for pulmonary hypertension in Philadelphia chromosome-negative myeloproliferative neoplasms

TL;DR

This study shows that myelofibrosis increases the risk of pulmonary hypertension in patients with Philadelphia chromosome-negative myeloproliferative neoplasms, which can worsen their prognosis.

Contribution

The study identifies myelofibrosis as a novel independent risk factor for pulmonary hypertension in Ph-MPN patients.

Findings

The overall prevalence of pulmonary hypertension in Ph-MPN patients was 15.4%.

Patients with myelofibrosis had a significantly higher risk of pulmonary hypertension (72.3%) compared to other Ph-MPN subtypes.

Secondary myelofibrosis was an independent risk factor for high-risk pulmonary hypertension.

Abstract

Philadelphia chromosome-negative myeloproliferative neoplasms (Ph-MPNs) are a group of malignant clonal disorders originating from bone marrow hematopoietic stem cells, and pulmonary hypertension (PH) is a serious progressive disease often coexisting with Ph-MPNs, with a prevalence ranging from 5 to 50%. The aim of this study was to analyze the prevalence, clinical characteristics, and associated risk factors of PH in 130 patients with Ph-MPNs, to investigate the impact of PH on patients’ prognosis, and to provide a reference for the clinical identification of adverse prognostic factors and early prevention and treatment of PH. One hundred and thirty patients with Ph-MPNs treated at Beijing Anzhen Hospital from January 1, 2020 to December 31, 2024 were included in the study. PH risk was assessed by echocardiography (ECHO), and tricuspid regurgitation velocity (TRV) > 2.8 m/s was used as the criterion for high risk of PH. General information, hematological indices, biochemical indices, gene mutations and echocardiographic data of the patients were collected and statistically analyzed. The overall prevalence of PH among the 130 patients with Ph-MPNs was 15.4%. patients with PMF had the highest prevalence of PH (72.3%), which was significantly higher than that of patients with PV (9.9%) and ET (10.4%) (P < 0.05). patients in the PH high-risk group were older, had lower hemoglobin levels, and had a higher prevalence of splenomegaly and secondary myelofibrosis. the PH high-risk group Patients had a significantly higher mortality rate than the normal risk group (20% vs. 1.81%, P = 0.0004). Multifactorial Cox regression analysis showed that advanced age (HR = 1.029, P = 0.0332) and high risk of PH (HR = 1.034, P = 0.0432) were independent risk factors for patient survival.Logistic regression analysis showed that decreased hemoglobin (OR = 0.9657, P = 0.0062), splenomegaly (OR = 5.105, P = 0.0413) and secondary myelofibrosis (OR = 7.959, P = 0.0321) were independent risk factors for high risk of PH. This study revealed the prevalence of PH, risk factors, and their prognostic implications in patients with Ph-MPNs. It is suggested that regular monitoring of changes in relevant risk factors and vigilance and prevention of PH during the treatment of patients with Ph-MPNs are clinically important to improve the prognosis of patients.

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