Peng Guangxin, Yang Wenrui, Zhang Li, Zhou Kang, Li Yang, Li Yuan, Ye Lei, Li Jianping, Fan Huihui, Song Lin, Zhao Xin, Wu Zhijie, Zhang Fengkui, Jing Liping
a Department of Anemia Therapeutic Centre , Institute of Hematology and Blood Diseases Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences (CAMS & PUMC) , Tianjin , China.
Hematology. 2016 Apr;21(3):138-43. doi: 10.1080/10245332.2015.1101977. Epub 2016 Mar 16.
T-cell large granular lymphocyte leukemia (T-LGLL) is a rare disorder characterized by clonal proliferation of large granular lymphocytes (commonly CD3+/CD8+/CD57+). However, the available data regarding the optimal treatment for relapsed/refractory T-LGLL patients are limited.
We retrospectively reviewed 10 patients treated with immunosuppressive therapy consisting of intravenous moderate-dose cyclophosphamide (MD-CTX) together with oral cyclosporine A for relapsed/refractory T-LGLL in our hospital between July 2006 and March 2013.
The overall response rate to MD-CTX was 60% (6/10; hematologic complete remission rate, 50%; hematologic partial remission rate, 10%). The median time to response was 28.5 days (range, 20-118 days). The relapse rate of MD-CTX was 50% (3/6); two of these three patients achieved hematologic complete remission after receiving a second course of MD-CTX. Neutropenia was the major adverse event of the MD-CTX regimen. The median time to neutropenia was 5.5 days (range, 1-10 days) and the median neutropenia duration was 5 days (range, 3-15 days). None of the patients developed severe infection.
The MD-CTX regimen appears efficacious and safe in the treatment of relapsed/refractory T-LGLL patients.
T 细胞大颗粒淋巴细胞白血病（T-LGLL）是一种罕见疾病，其特征为大颗粒淋巴细胞（通常为 CD3+/CD8+/CD57+）的克隆性增殖。然而，关于复发/难治性 T-LGLL 患者最佳治疗方法的现有数据有限。
我们回顾性分析了 2006 年 7 月至 2013 年 3 月期间在我院接受免疫抑制治疗（由静脉注射中等剂量环磷酰胺（MD-CTX）联合口服环孢素 A 组成）的 10 例复发/难治性 T-LGLL 患者。
MD-CTX 的总体缓解率为 60%（6/10；血液学完全缓解率为 50%；血液学部分缓解率为 10%）。缓解的中位时间为 28.5 天（范围为 20 - 118 天）。MD-CTX 的复发率为 50%（3/6）；这 3 例患者中有 2 例在接受第二个疗程的 MD-CTX 后实现了血液学完全缓解。中性粒细胞减少是 MD-CTX 方案的主要不良事件。中性粒细胞减少的中位时间为 5.5 天（范围为 1 - 10 天），中性粒细胞减少的中位持续时间为 5 天（范围为 3 - 15 天）。所有患者均未发生严重感染。
MD-CTX 方案在治疗复发/难治性 T-LGLL 患者中似乎有效且安全。
