较高的JAK2V617F等位基因负担可能是年轻的多发性红细胞症患者发生出血事件的风险因素。

IF 2 4区医学 Q3 HEMATOLOGY

Hematology Pub Date : 2024-12-01 Epub Date: 2024-11-15 DOI:10.1080/16078454.2024.2427905

Chiho Furuya, Yoshinori Hashimoto, Soji Morishita, Yasutaka Fukuda, Tadaaki Inano, Tomonori Ochiai, Shuichi Shirane, Yoko Edahiro, Marito Araki, Miki Ando, Norio Komatsu

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引用次数: 0

摘要

目的：出血事件是多血症患者罕见但可能致命的并发症：出血事件是红细胞增多症（PV）患者罕见但可能致命的并发症：我们分析了267名红细胞增多症患者出血事件的特征：结果：中位随访 4.8 年，共发现 23 例（8.6%）出血事件。60岁以下的年轻患者（n = 72）发生的出血事件明显多于60岁或以上的老年患者（n = 191）（分别为n = 12 [16.7%] vs. n = 11 [5.8%]，P = 0.012）。在年轻患者的单变量分析中，白细胞（WBC）计数≥ 15 × 109/L（危险比 [HR] = 7.746，95% 置信区间 [CI] 2.082-28.830，P = 0.002）、可触及脾肿大（HR = 5.629，95% CI 1.193-26.550，P = 0.029）、JAK2V617F 等位基因负荷≥80%（HR = 22.850，95% CI 2.885-181.00，P = 0.003）与出血事件风险增加相关。在多变量分析中，JAK2V617F等位基因负荷≥80%（HR = 9.394，95% CI 1.046-84.380，P = 0.046）是一个重要的风险因素：JAK2V617F等位基因负荷高、白细胞计数高或可触及脾肿大的年轻PV患者确诊后发生出血事件的风险增加。重要的是要考虑治疗方案，旨在通过降低年轻 PV 患者的 JAK2V617F 等位基因负荷来避免出血事件的发生。

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A higher JAK2V617F allele burden may be a risk factor for hemorrhagic events in younger patients with polycythemia vera.

Objectives: Hemorrhagic events are a rare but potentially fatal complication in patients with polycythemia vera (PV).

Methods: We analyzed the characteristics of hemorrhagic events in 267 patients with PV.

Results: A median follow-up of 4.8 years revealed that 23 (8.6%) hemorrhagic events occurred. Significantly more hemorrhagic events occurred in younger patients aged below 60 years (n = 72) than in older patients aged 60 years or above (n = 191) (n = 12 [16.7%] vs. n = 11 [5.8%], respectively, P = 0.012). In univariate analysis among the younger patients, white blood cell (WBC) count ≥ 15 × 10⁹/L (hazard ratio [HR] = 7.746, 95% confidence interval [CI] 2.082-28.830, P = 0.002), palpable splenomegaly (HR = 5.629, 95% CI 1.193-26.550, P = 0.029), and JAK2V617F allele burden ≥ 80% (HR = 22.850, 95% CI 2.885-181.00, P = 0.003) were associated with an increased risk of hemorrhagic events. In multivariate analysis, JAK2V617F allele burden ≥ 80% (HR = 9.394, 95% CI 1.046-84.380, P = 0.046) was a significant risk factor.

Conclusions: There is an increased risk of hemorrhagic events after diagnosis in younger PV patients with a high JAK2V617F allele burden, high WBC count or palpable splenomegaly. It is important to consider treatment options that aim to avoid hemorrhagic events by reducing the JAK2V617F allele burden in younger PV patients.

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来源期刊

Hematology 医学-血液学

CiteScore

2.60

自引率

5.30%

发文量

140

审稿时长

3 months

期刊介绍： Hematology is an international journal publishing original and review articles in the field of general hematology, including oncology, pathology, biology, clinical research and epidemiology. Of the fixed sections, annotations are accepted on any general or scientific field: technical annotations covering current laboratory practice in general hematology, blood transfusion and clinical trials, and current clinical practice reviews the consensus driven areas of care and management.