CAJ | 학술논문

目的观察利妥昔单抗(RTX)在特发性血栓性血小板减少性紫癜(ITTP)治疗中的价值.方法 17例ITTP患者中9例采用RTX+血浆置换+糖皮质激素治疗(RTX组),8例采用血浆置换+糖皮质激素±免疫抑制剂(对照组).RTX用法为375mg/m2每周1次连续4次.监测患者血常规、LDH、ADAMTS 13活性及抑制物、外周血B淋巴细胞百分比.观察两组患者的血浆置换次数/总量、缓解时间、复发率及不良反应发生情况.结果 RTX组、对照组血浆置换次数中位数分别为5(2～8)、6(4～9)次,血浆置换总量中位数分别为9.6(4.0～15.4)、11.2(7.5～14.6)L.RTX组、对照组达到血液学缓解中位时间分别为15(5～20)d、22(7～36)d,达到免疫学缓解中位时间分别为2(2～8)、2(2～4)周.两组患者ADAMTS 13活性均在2周后显著回升.RTX组随访期内无复发,对照组4例患者复发.RTX给药前及治疗后1周患者外周血B淋巴细胞百分比差异有统计学意义[(18.39±7.15)％对(2.19±5.11)％,P＜0.001],9个月后逐渐回升,治疗中未观察到RTX相关严重不良反应,但该组1例患者持续免疫学缓解7个月后死于肺部感染.结论 RTX联合血浆置换和糖皮质激素治疗ITTP,能快速获得血液学甚至免疫学缓解,降低复发率,不良反应少,但需注意防治感染.
목적 관찰리타석단항(RTX)재특발성혈전성혈소판감소성자전(ITTP)치료중적개치.방법 17례ITTP환자중9례채용RTX+혈장치환+당피질격소치료(RTX조),8례채용혈장치환+당피질격소±면역억제제(대조조).RTX용법위375mg/m2매주1차련속4차.감측환자혈상규、LDH、ADAMTS 13활성급억제물、외주혈B림파세포백분비.관찰량조환자적혈장치환차수/총량、완해시간、복발솔급불량반응발생정황.결과 RTX조、대조조혈장치환차수중위수분별위5(2～8)、6(4～9)차,혈장치환총량중위수분별위9.6(4.0～15.4)、11.2(7.5～14.6)L.RTX조、대조조체도혈액학완해중위시간분별위15(5～20)d、22(7～36)d,체도면역학완해중위시간분별위2(2～8)、2(2～4)주.량조환자ADAMTS 13활성균재2주후현저회승.RTX조수방기내무복발,대조조4례환자복발.RTX급약전급치료후1주환자외주혈B림파세포백분비차이유통계학의의[(18.39±7.15)％대(2.19±5.11)％,P＜0.001],9개월후축점회승,치료중미관찰도RTX상관엄중불량반응,단해조1례환자지속면역학완해7개월후사우폐부감염.결론 RTX연합혈장치환화당피질격소치료ITTP,능쾌속획득혈액학심지면역학완해,강저복발솔,불량반응소,단수주의방치감염.
Objective To study the efficacy and safety of rituximab (RTX) in the treatment of idiopathic thrombotic thrombocytopenic purpura (ITTP).Methods 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.Results 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.Conclusion 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.