口服环磷酰胺治疗难治性T细胞大颗粒淋巴细胞白血病合并纯红细胞再生障碍性贫血的疗效

目的:探讨小剂量环磷酰胺口服治疗难治性T-大颗粒淋巴细胞白血病(T-LGLL)伴纯红细胞再生障碍性贫血(PRCA)的疗效。方法:报告1例T-LGLL合并PRCA患者的临床表现、实验室检查特征,及应用环孢素A联合强的松无效后给予口服小剂量环磷酰胺的治疗经过,并结合文献进行讨论。结果:患者为老年女性,临床进展缓慢,以贫血为主要表现,骨髓红系细胞增生显著低下,血涂片以大颗粒淋巴细胞为主,免疫分型及基因重排符合T-LGLL,应用环孢素A联合强的松治疗5个月无效,需要依赖反复输血。二线改用环磷酰胺100 mg/d口服,血红蛋白恢复至正常水平,停药1年仍维持疗效。结论:T-LGLL合并PRCA少见,小剂量环磷酰胺可作为有效的治疗方案。

Efficacy of Oral Cyclophosphamide for Treatment of Refractory T-cell Large Granular Lymphocytic Leukemia Accompanied with Pure Erythrocyte Aplastic Anemia

Objective:To explore the clinical effects of oral small dose of cyclophosphamide(CTX)in the treatment of T-cell large granular lymphocytic leukemia(T-LGLL)accompanied with pure red cell aplasia(PRCA).Methods:The clinical features,characteristics of laboratory examinations and the process of oral small dose of CTX treatment after the ineffective treatment of cyclosporine A combining with prednisone in 1 case of T-LGLL with PRCA were reported and discussed with related references.Results:The elderly female patient had indolent process,mainly presenting with anemia and significant low hyperplasia of bone marrow erythrocyte cells.Peripheral blood smear showed mainly with characteristic large granular lymphocytic morphology.The results of immunophenotypic analyses and genetic reassortment were compatible with T-LGLL.No effects were shown after 5 months of cyclosporine A combining with prednisone treatment and the patient still needed recurrent blood transfusion.CTX was prescribed as a second-line medication and the dose was 100 mg/d.Hemoglobin could returned to normal level and the efficacy remained for 1 year even after the medication was stopped.Conclusion:T-LGLL accompanied with PRCA is a rare disease and oral small dose CTX can be an effective therapeutic regimen.