Wang Jing, Wu Tianqin, Shen Hongshi, Ren Chuanlu, Chen Haifei, She Ziqiang, Wang Zhaoyue
Department of Hematology, 100th Hospital of PLA, Suzhou 215007, China.
Zhonghua Xue Ye Xue Za Zhi. 2015 Apr;36(4):316-20. doi: 10.3760/cma.j.issn.0253-2727.2015.04.012.
To study the efficacy and safety of rituximab (RTX) in the treatment of idiopathic thrombotic thrombocytopenic purpura (ITTP).
Among 17 ITTP patients, nine cases of the RTX group were administrated with RTX plus plasma exchange (PEX) and steroids. Eight cases of the control group received PEX plus steroids±other immune inhibitors. Patients received RTX 375 mg/m², 1 per week for 4 weeks. The laboratory parameters, including hemogram, LDH, ADAMTS13 activities and its inhibitors, and the ratio of B lymphocytes in peripheral blood were monitored. The number of PEX, total plasma volumes, remission time, relapse ratio and adverse effects in both groups were compared.
The median number of PEX/median total plasma volumes in the RTX and control group were 5(2-8)/9.6(4.0-15.4) L and 6(4-9)/11.2(7.5-14.6) L, respectively. Patients in the RTX and control group achieved hematologic remission at the median time of 15(5-20) days and 22(7-36) days, respectively. And the median time of immunological remission in the two groups was 2(2-8) and 2(2-4) weeks, respectively. ADAMTS13 activities increased significantly after 2 weeks in both two groups. There was no relapse in the RTX group, while 4 patients relapsed in the control group. The percentage of B lymphocytes in peripheral blood obviously deduced one week after first dose of RTX infusion compared with the level before treatment [(2.19±5.11)% vs (18.39±7.15)%, P<0.001], and began to gradually increase 9 months later. Severe adverse events were not observed in RTX group during the therapeutic procedure and follow-up, but one patient, who had sustained immunologic remission, died of severe pneumonia 7 months later.
In the treatment of ITTP, RTX in conjunction with PEX and steroids appeared to be a safe and effective therapy, with fast and sustained remission in hematology and even in immunology, with lower relapse rate and less adverse effects. But patients needed to be paid attention to prevent and treat infectious events in time.
探讨利妥昔单抗（RTX）治疗特发性血栓性血小板减少性紫癜（ITTP）的疗效及安全性。
17例ITTP患者中，RTX组9例接受RTX联合血浆置换（PEX）及糖皮质激素治疗。对照组8例接受PEX联合糖皮质激素±其他免疫抑制剂治疗。患者接受RTX 375mg/m²，每周1次，共4周。监测血常规、乳酸脱氢酶（LDH）、ADAMTS13活性及其抑制剂、外周血B淋巴细胞比例等实验室指标。比较两组的PEX次数、总血浆量、缓解时间、复发率及不良反应。
RTX组和对照组的PEX次数中位数/总血浆量中位数分别为5（2 - 8）次/9.6（4.0 - 15.4）L和6（4 - 9）次/11.2（7.5 - 14.6）L。RTX组和对照组患者达到血液学缓解的中位时间分别为15（5 - 20）天和22（7 - 36）天。两组免疫缓解的中位时间分别为2（2 - 8）周和2（2 - 4）周。两组在2周后ADAMTS13活性均显著升高。RTX组无复发，而对照组有4例复发。首次输注RTX 1周后外周血B淋巴细胞百分比与治疗前水平相比明显下降[（2.19±5.11）% vs（18.39±7.15）%，P<0.001]，9个月后开始逐渐升高。RTX组在治疗过程及随访期间未观察到严重不良事件，但1例持续免疫缓解的患者7个月后死于重症肺炎。
在ITTP治疗中，RTX联合PEX及糖皮质激素似乎是一种安全有效的治疗方法，血液学甚至免疫学缓解快速且持久，复发率较低，不良反应较少。但需注意及时预防和治疗感染事件。
