异基因造血干细胞移植后慢性移植物抗宿主病相关性肾病综合征

Nephrotic Syndrome associated with Chronic Graft-versus-host Disease follow-ing Allogeneic Stem Cell Transplantation:A Case Report and Literature Review

目的探讨异基因造血干细胞移植(allo-HSCT)后慢性移植物抗宿主病(cGVHD)相关性肾病综合征(NS)的临床特点及治疗方法。方法对1例急性髓性白血病M2(AML-M2)的患者采用了改良的马利兰/环磷酰胺(Bu/Cy)预处理方案进行了allo-HSCT。移植后8月出现骨髓细胞学复发,给予化疗及两次供者淋巴细胞输注(DLI),回输的单个核细胞(MNC)数分别为6.80×107/kg和8.70×107/kg,第二次DLI后2月患者出现双下肢凹陷性水肿,呈进行性加重,同时伴有口干、眼干的症状,查ALT 263U/L,AST 89U/L,黄疸指数正常,肾功能正常,24h尿蛋白定量4.4g,24h尿白蛋白定量4.15g,血清白蛋白为27.4g/L,胆固醇总量为7.48mmol/L,甘油三酯为3.6mmol/L。抗核抗体(ANA)1:1000,颗粒型,抗U1RNP抗体弱阳性。考虑为cGVHD相关的NS。予硫唑嘌呤、环孢素A和泼尼松治疗,同时给予补充白蛋白、利尿等。结果患者于治疗2月后尿蛋白逐渐转阴,口干、眼干的症状改善,24h尿蛋白定量下降至0.15g,24h尿白蛋白定量下降至0.13g,血清白蛋白上升至38.5g/L,血脂正常,ANA滴度下降至1:100,抗U1RNP抗体转阴。随访到目前移植后22月,第二次DLI后11月,患者尿蛋白持续阴性,骨髓处于持续完全缓解状态,仍在继续随访中。结论cGVHD相关的NS,应尽量行肾组织活检,取得其病理诊断,以便进行相应的治疗。

Objective To study clinical features of nephrotic syndrome （NS） associated with chronic graftversus-host disease （cGVHD） following allogeneic hematopoietic stem cell transplantation （allo-HSCT）,and to explore its treatment.Methods 1 patient with AML-M2 received improved busulfan / cyclophosphamide regimens.The relapse of medulla occurred in the 8th month post-transplantation,and the combination of chemical treatment and twi-donor lymphocyte infusion（DLI） were received.The cell dose respectively were 6.80 × 10^7MNC/kg and 8.70 × 10^7MNC/kg.The hollow edema of both crura occurred in the the 2nd month post-second DLI, and it aggravated gradually. It went with dry eyes and mouth coinstantaneously. The patient got ALT 263U/L, AST 89U/L, 24h urine protein 4.4g, 24-h urine albumin 4.15g, serum albumin 27.4g/L,total cholesterol 7.48 mmol/L,triglycedde 3.6 mmol/ L, antinuclear antibody（ANA） 1：1000 （granula）,antiU1RNP antibody weak-positive.The diagnosis of the patient was NS associated with cGVHD and treated with azathiopdne,cyclospodne A, prednisone, supply of albumin and diuresis. Results After treatment for 2 months, proteinuria was gradually decreased and the symptoms of dry eyes and mouth were improved.The 24-h urine protein and urine albumin declined to 0.15g and 0.13g.The serum albumin ascended to 38.5g/L and the serum lipid was normal.The ANA declined to 1：100 and antiU 1RNP antibody changed into negative.The patient was followed for 22 months after alIo-HSCT and 11 months after second DLI, and the function ofhemopoiesis was well. The state of illness retained continuous remission.Conclusions The patient with NS associated with cGVHD should receive renal biopsy and get its pathological diagnosis, and it benefits the treatment.