Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Department of Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Front Immunol. 2024 Oct 16;15:1464616. doi: 10.3389/fimmu.2024.1464616. eCollection 2024.
Chronic graft-versus-host disease (GVHD) is a major complication after allogeneic hematopoietic stem cell transplantation (HSCT). Chronic GVHD may have atypical manifestations affecting non-classical organs. The diagnosis in patients with atypical manifestations of chronic GVHD is particullarly challenging, and there is a lack of knowledge regarding their pathogenesis and treatment. We reported a case who developed post-HSCT nephrotic syndrome and portal hypertensive ascites, which are both rare and atypical manifestations of chronic GVHD. Kidney biopsy revealed membranous nephropathy and renal thrombotic microangiopathy with glomerular immune deposits, suggesting antibody-mediated kidney injury. Treatment with ruxolitinib resulted in remission of both nephrotic syndrome and ascites, suggesting a role of cytokines in the pathogenesis. This case highlighted the awareness of nephrotic syndrome and portal hypertensive ascites as atypical manifestations of chronic GVHD, and the efficacy of ruxolitinib for the two manifestations.
慢性移植物抗宿主病（GVHD）是异基因造血干细胞移植（HSCT）后的主要并发症。慢性 GVHD 可能具有影响非典型器官的非典型表现。具有慢性 GVHD 非典型表现的患者的诊断特别具有挑战性，并且对其发病机制和治疗方法缺乏了解。我们报告了一例患者在 HSCT 后发生肾病综合征和门脉高压性腹水，这两种都是慢性 GVHD 的罕见和非典型表现。肾脏活检显示膜性肾病和伴有肾小球免疫沉积物的肾血栓性微血管病，提示抗体介导的肾损伤。用芦可替尼治疗使肾病综合征和腹水均缓解，提示细胞因子在发病机制中的作用。该病例强调了将肾病综合征和门脉高压性腹水作为慢性 GVHD 的非典型表现的认识，以及芦可替尼对这两种表现的疗效。
