Department of Nephrology and Immunology, Children's Hospital of Soochow University, Suzhou, Jiangsu, China.
Department of Hematology, Children's Hospital of Soochow University, Suzhou, Jiangsu, China.
BMC Pediatr. 2023 Apr 21;23(1):186. doi: 10.1186/s12887-023-03996-1.
Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative therapy for hematologic malignancies and non-malignant disorders, such as aplastic anemia, fanconi anemia, and certain immune deficiencies. Post-transplantation kidney injury is a common complication and involves a wide spectrum of structural abnormalities, including glomerular (MSPGN, mesangial proliferative glomerulonephritis; FSGS, focal segmental glomerulosclerosis; MPGN, membranoproliferative glomerulonephritis; MCD, minimal change disease), vascular (TMA, thrombotic microangiopathy), and/or tubulointerstitial (TIN, tubulointerstitial nephritis; ATI, acute tubular injury). Renal biopsy is the gold-standard examination for defining multiple etiologies of kidney impairment. Although kidney injury following HSCT has been studied, little is known about the effects of allo-HSCT on renal pathology in pediatric patients.
We retrospectively analyzed renal biopsy specimens from children with kidney injury after allo-HSCT and correlated results with clinical data in the last 10 years.
Among 25 children (18 males and 7 females), three patients had proteinuria indicating nephrotic syndrome (24-hour urinary total protein/weight > 50 mg/kg/d), nine patients had severely reduced estimated glomerular filtration rate (eGFR < 30 ml/min/1.73 m) and four patients received kidney replacement therapy (KRT). The main pathologies identified from kidney biopsies were MSPGN (n = 12), FSGS (n = 12), MPGN (n = 5), TMA (n = 4), MCD (n = 3), diffuse glomerular fibrosis (DGF, n = 2), ATI and TIN, in isolation or combined with other pathologies. The median follow-up time was 16.5 (0.5 ~ 68.0) months. Three patients died of recurrent malignancy and/or severe infection, one child developed to end-stage renal disease (ESRD), six patients (24%) had elevated serum creatinine (SCr > 100µmol/l) and nine patients (36%) still had proteinuria.
This study evaluates histomorphologic findings from kidney biopsies of pediatric recipients following allo-HSCT. Detailed evaluation of renal biopsy samples is helpful to elucidate the nature of renal insult, and may potentially identify treatable disease processes.
异基因造血干细胞移植（allo-HSCT）是治疗血液系统恶性肿瘤和非恶性疾病的一种有治愈可能的疗法，例如再生障碍性贫血、范可尼贫血和某些免疫缺陷。移植后肾脏损伤是一种常见的并发症，涉及广泛的结构异常，包括肾小球（MSPGN、系膜增生性肾小球肾炎；FSGS、局灶节段性肾小球硬化；MPGN、膜增生性肾小球肾炎；MCD、微小病变病）、血管（TMA、血栓性微血管病）和/或肾小管间质（TIN、肾小管间质性肾炎；ATI、急性肾小管损伤）。肾脏活检是确定肾脏损伤多种病因的金标准检查。尽管已经研究了 HSCT 后肾脏损伤，但对于 allo-HSCT 对儿科患者肾脏病理的影响知之甚少。
我们回顾性分析了 10 年来接受 allo-HSCT 后发生肾脏损伤的儿童的肾脏活检标本，并将结果与临床数据相关联。
在 25 名儿童（18 名男性和 7 名女性）中，3 名患者出现蛋白尿提示肾病综合征（24 小时尿总蛋白/体重>50mg/kg/d），9 名患者肾小球滤过率严重降低（eGFR<30ml/min/1.73m），4 名患者接受肾脏替代治疗（KRT）。肾脏活检的主要病理改变为 MSPGN（n=12）、FSGS（n=12）、MPGN（n=5）、TMA（n=4）、MCD（n=3）、弥漫性肾小球纤维化（DGF，n=2）、孤立或合并其他病理改变的 ATI 和 TIN。中位随访时间为 16.5（0.5~68.0）个月。3 名患者死于复发性恶性肿瘤和/或严重感染，1 名儿童发展为终末期肾病（ESRD），6 名患者（24%）血清肌酐（SCr>100μmol/l）升高，9 名患者（36%）仍有蛋白尿。
本研究评估了 allo-HSCT 后儿科受者肾脏活检的组织形态学发现。详细评估肾脏活检样本有助于阐明肾脏损伤的性质，并可能确定可治疗的疾病过程。
