毛细胞白血病的预后:单中心研究

IF 0.1 Q4 HEMATOLOGY
Hana Kamal Yalda Kaka, Ahmed K. Yassin, Kawa Muhamedamin Hasan
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引用次数: 0

摘要

毛细胞白血病(HCL)是一种罕见的慢性 B 细胞淋巴增生性疾病。使用嘌呤类似物和免疫疗法的现代疗法可使病情得到长期缓解,但复发风险仍高达40%-50%。本研究旨在评估在纳纳卡利医院接受治疗的 HCL 患者的疗效。 本研究对伊拉克库尔德地区埃尔比勒市纳纳卡利医院 2004 年至 2022 年期间确诊的 50 名 HCL 患者进行了回顾性横断面研究。研究人员从病历中收集了患者的人口统计学特征、临床表现、治疗数据、并发症、反应、复发和存活数据。结果采用描述性统计。变量比较采用卡方分析。 平均年龄为(52.64±12.37)岁,84%为男性。最常见的症状是脾肿大(18%)和乏力(14%)。大多数患者(69.6%)接受了克拉利宾治疗;应答率为 73.9%,其中完全缓解率为 67.4%。47.8%的患者病情复发。最常见的不良反应是发热性中性粒细胞减少(58.7%)和 III 级和 IV 级血液学毒性(41.3%)。结果与治疗前的ANC(P = 0.019)、合并症(P = 0.001)和治疗反应(P = 0.004)明显相关。克拉利宾-利妥昔单抗联合治疗可获得完全缓解(100%)。十年总生存率为 70%。 研究结果与文献报道基本一致,证明了克拉利宾联合/不联合利妥昔单抗作为HCL一线疗法的有效性和安全性,但30%的死亡率令人担忧。进一步的研究应找出影响亚组不良预后的可改变因素,以指导改善HCL的风险管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Outcome of Hairy Cell Leukemia: A Single-center Study
Hairy cell leukemia (HCL) is a rare chronic B-cell lymphoproliferative disorder. Modern therapy with purine analogs and immunotherapy can provide long-term remission, but the risk of recurrence remains about 40%–50%. The aim of this study was to evaluate the outcome of patients with HCL who received treatment in Nanakali Hospital. A retrospective cross-sectional study was carried out on 50 patients of HCL diagnosed from 2004 to 2022 in Nanakali Hospital in Erbil City, Kurdistan Region, Iraq. Demographics, clinical presentation, treatment data, complications, response, recurrence, and survival data were collected from medical records. The results were presented with descriptive statistics. Variables were compared by Chi-square analysis. The mean age was 52.64 ± 12.37 years, and 84% were male. The most common presenting symptoms were splenomegaly (18%) and fatigue (14%). The majority (69.6%) received cladribine; the response rate was 73.9%, with a complete remission (67.4%). 47.8% had recurrent disease. The most common adverse effects were febrile neutropenia (58.7%) and Grade III and IV hematologic toxicity (41.3%). The results were significantly associated with ANC pretreatment (P = 0.019), comorbidity (P = 0.001), and treatment response (P = 0.004). Cladribine–rituximab combination resulted in complete remission (100%). Ten-year overall survival was 70%. The results were broadly consistent with literature reports, demonstrating the efficacy and safety of cladribine with/without rituximab as first-line therapy for HCL but with a 30% mortality of concern. Further studies should identify modifiable factors that affect poor prognosis in subgroups to guide improvements in risk management of HCL.
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