Iijima Kimiko, Hirao Masako, Hino Toshiya, Kamoda Yoshimasa, Iizuka Hiromitsu, Kida Michiko, Hangaishi Akira, Usuki Kensuke
Department of Hematology, NTT Medical Center Tokyo.
Rinsho Ketsueki. 2017;58(12):2392-2396. doi: 10.11406/rinketsu.58.2392.
A 39-year-old man with anemia presented at our hospital in November 2011. Peripheral blood analysis revealed lymphocytosis with a large granular lymphocyte (LGL) count of 2,272/µl, with CD3+, CD4-, CD8+, CD56-, TCR-αβ+; Southern blotting analysis revealed clonal TCR Cβ 1 gene rearrangement, leading to the diagnosis of T-LGL leukemia. In June 2012, the patient was administered with cyclophosphamide as an initial treatment because he developed transfusion-dependent anemia. His anemia improved, and the treatment was discontinued in March 2013. However, anemia recurred in March 2014. The administration of cyclophosphamide was resumed; however, it was subsequently replaced with cyclosporine because of the risk of secondary cancer due to the long-term use of cyclophosphamide. However, his anemia did not improve. Further, the patient was administered with prednisone, methotrexate, and pentostatin; however, the transfusion-dependent state persisted with the cumulative transfusion of 186 RBC units until March 2016. After CD52 expression on the surface of LGL cells was confirmed, treatment with alemtuzumab, which is a monoclonal antibody against CD52, was initiated in April 2016 and the dose was gradually increased from 3 mg to 30 mg thrice per week. The patient's anemia began to improve 1 week after initiating alemtuzumab treatment, and he became transfusion-independent in the second week. Although alemtuzumab treatment was discontinued at the fifth week on the basis of a positive test result for CMV antigenemia, the result consequently became negative after ganciclovir treatment. To date, the patient's hemoglobin level has been maintained at approximately 12 g/dl without any treatment. Herein we reported the case of a patient having LGL leukemia with refractory anemia that was successfully treated using alemtuzumab.
一名39岁的贫血男性于2011年11月到我院就诊。外周血分析显示淋巴细胞增多,大颗粒淋巴细胞(LGL)计数为2272/µl,CD3+、CD4-、CD8+、CD56-、TCR-αβ+;Southern印迹分析显示克隆性TCR Cβ 1基因重排,从而诊断为T-LGL白血病。2012年6月,该患者因出现输血依赖型贫血而接受环磷酰胺作为初始治疗。其贫血症状有所改善,治疗于2013年3月停止。然而,2014年3月贫血复发。于是恢复环磷酰胺治疗;但由于长期使用环磷酰胺有继发癌症的风险,随后改用环孢素。然而,他的贫血并未改善。此外,该患者还接受了泼尼松、甲氨蝶呤和喷司他丁治疗;但直到2016年3月,输血依赖状态持续存在,累计输注红细胞186单位。在确认LGL细胞表面有CD52表达后,2016年4月开始使用抗CD52单克隆抗体阿仑单抗进行治疗,剂量从3 mg逐渐增加至每周三次30 mg。开始阿仑单抗治疗1周后患者的贫血开始改善,第二周就不再依赖输血。尽管基于CMV抗原血症检测结果呈阳性,在第五周停止了阿仑单抗治疗,但在更昔洛韦治疗后结果转为阴性。迄今为止,患者的血红蛋白水平在未进行任何治疗的情况下一直维持在约12 g/dl。在此我们报告了一例患有LGL白血病伴难治性贫血的患者,使用阿仑单抗成功治愈的病例。