Purpose

To assess the prevalence of PHT in patients with BCR-ABL1–negative CMPN and to evaluate impact of PHT on survival during long-term follow-up.

Patients and Methods

A total of 122 patients with BCR-ABL1–negative CMPN underwent transthoracic echocardiographic (TTE) evaluation at the beginning of study. Patients undergoing PHT on TTE examination were also evaluated by a pulmonologist. Patients were divided into 3 groups. Group A comprised patients with CMPN-related PHT; group B, patients with no PHT; and group C, patients with PHT due to secondary causes. Patients were evaluated again every 3 to 6 months.

Results

PHT was detected in 33 (27%) of 122 patients. Eight (6.5%) had CMPN-related PHT and the remaining 25 (20.5%) had non–CMPN-related PHT. Positivity for JAK2 V617F mutation in the study population was 72.9%. Groups were similar with respect to hematologic parameters and gender. Follow-up times were as follows: median (range) time from diagnosis to TTE and study end were 34 (1-158) months and 107 (16-251) months, respectively, and from TTE to study end was 88 (7-110) months. No significant differences found among the groups in terms of median time from diagnosis to TTE, follow-up, and overall survival.

Conclusion

BCR-ABL1–negative CMPN patients had a lower prevalence of PHT compared to earlier studies. There was no statistically significant difference in median overall survival between patients with or without PHT. This may be because patients with PHT were asymptomatic and PHT was mild. The impact of PHT on survival was negligible.

中文翻译：

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BCR-ABL1-阴性慢性骨髓增生性肿瘤和肺动脉高压：肺动脉高压对生存影响的前瞻性长期随访研究

目的

评估BCR-ABL1阴性 CMPN患者 PHT 的患病率，并评估 PHT 对长期随访期间生存率的影响。

患者和方法

共有 122 名BCR-ABL1阴性 CMPN 患者在研究开始时接受了经胸超声心动图 (TTE) 评估。在 TTE 检查中接受 PHT 的患者也由肺科医生进行评估。患者被分为3组。A 组包括 CMPN 相关 PHT 患者；B组，无PHT患者；C组为继发性PHT患者。每 3 至 6 个月对患者进行一次评估。

结果

在 122 名患者中的 33 名 (27%) 中检测到 PHT。8 人 (6.5%) 患有 CMPN 相关 PHT，其余 25 人 (20.5%) 患有非 CMPN 相关 PHT。阳性的JAK2研究人群V617F突变为72.9％。各组在血液学参数和性别方面相似。随访时间如下：从诊断到 TTE 和研究结束的中位（范围）时间分别为 34 (1-158) 个月和 107 (16-251) 个月，从 TTE 到研究结束的时间为 88 (7-251) 个月。 110) 个月。在从诊断到 TTE 的中位时间、随访和总生存期方面，各组之间没有发现显着差异。

结论

与早期研究相比，BCR-ABL1阴性 CMPN 患者的 PHT 患病率较低。有或没有 PHT 的患者的中位总生存期没有统计学上的显着差异。这可能是因为 PHT 患者无症状且 PHT 较轻。PHT对生存的影响可以忽略不计。