小剂量利妥昔单抗治疗原发性干燥综合征继发血小板减少四例疗效观察

The efficacy and safety of low-dose rituximab in treatment of primary Sj^gren＇s syndrome withthrombocytopenia

目的初步评价小剂量利妥昔单抗治疗原发性干燥综合征（pSS）继发血小板减少的疗效与安全性。方法4例pSS继发血小板减少患者，2例为难治性血小板减少，2例为糖皮质激素依赖性血小板减少，静脉滴注利妥昔单抗100nag，每周1次，共2次，同时联合泼尼松1-2mg·kg-1·d“治疗。观察血小板和外周血B细胞的变化。结果4例患者治疗前血小板水平为（3—39）×109／L，d,N量利妥昔单抗治疗后，血小板于1-2周内上升，3—8周内恢复至（107-241）x109／L，维持缓解27-52周。12周内泼尼松减为3．75-7．50mg／d并维持。1例患者于第27周时复发，血小板降至47×10-／L，再次静脉滴点利妥昔单抗100mg，4周后血小板升至81x10-／L。4例患者外周血B细胞降至（0．007-0．010）×10-／L，但未达清除状态。输注过程中均无严重不良反应发生。结论小剂量利妥昔单抗可用于治疗pSS继发血小板减少，减少糖皮质激素用量，部分清除B细胞。

Objective To investigate the clinical efficacy and safety of low-dose rituximab （RTX） for patients in primary SjSgren＇s syndrome （pSS） with thrombocytopenia. Methods Four pSS patients, 2 with refractory thrombocytopenia and 2 with glucocorticoid-dependent thrombocytopenia, were treated with rituximab at 100 mg, intravenous, weekly for a total of two cycles, together with prednisone 1-2 mg · kg -1· d -1 , and the counts of platelets and B-cells were evaluated. Results Efficacy of treatment was observed in all patients. The counts of platelets, at （3-39） x 109/L baseline, incleased in 1-2 weeks, and went up to （107-241） x 109/L in 3-8 weeks. Sustained remission had been achieved for 27-52 weeks. The doses of prednisone were tappered to 3.75-7.50 rag/day in 12 weeks. One patient who relapsed at the 27th week （platelet count 47 x 109/L） , was retreated with 100 mg of RTX and still had good efficacy. The counts of B-cells reduced to （0. 007-0. 010） x 109/L, but they did not achieved the depletion. There were no severe adverse events during RTX therapy. Conclusions Our study has shown good efficacy and tolerability of low- dose RTX for pSS with thrombocytopenia. Low-dose RTX allows for reduction in corticosteroid doses and B-cells, while large-scale randomized double-blind controlled trials are needed to confirm the results.