John Elenjickal Elias, Roy Sanjeet, Devasia Anup J, Karuppusami Reka, Jose Nisha, Mani Selvin Sundar Raj, Eapen Jeethu Joseph, Yusuf Sabina, Thomas Athul, Valson Anna T, David Vinoi George, Mathews Vikram, Varughese Santosh, Alexander Suceena
Department of Nephrology, Christian Medical College, Vellore, India.
Department of Pathology, Christian Medical College, Vellore, India.
Glomerular Dis. 2023 Mar 14;3(1):98-115. doi: 10.1159/000529699. eCollection 2023 Jan-Dec.
Post hematopoietic stem cell transplant (HSCT), kidney can be subjected to injury by various causes. Of these, graft versus host disease (GvHD) affecting the kidney is an under-recognized entity with no clear guidelines on its diagnosis, clinicopathological manifestations, and outcomes.
Out of 2,930 patients who underwent HSCT at our center between 2005 and 2020, kidney biopsy was performed in 19 allogenic and 5 autologous recipients.
The mean age of the cohort at transplant was 33.2 ± 7 years, and 15 (62%) were males. Median time to kidney biopsy from HSCT was 14 (IQR, 9-30) months. Aplastic anemia was the most common underlying hematological disease (54.2%). All 19 allogenic recipients were classified based on clinicopathological manifestations into either thrombotic microangiopathy (TMA, 12/19 [63%]) or nephrotic syndrome (NS, 7/19 [37%]) pattern. Glomerular tuft "mesangiolysis" was the dominant pattern of injury noted in 9/12 cases of TMA pattern. There was a predominance of acute microangiopathic changes restricted primarily to the glomerular compartment. Of the 7 patients with NS pattern, membranous nephropathy was seen in 4 (57%) and minimal change disease in 3 (43%) patients. Thirty-nine percent (7/18) stained positive for C4d which was predominantly glomerular. Allogenic recipients who did not receive immunosuppression (IS) for renal disease had a lower eGFR at biopsy, a longer latency between withdrawal of GvHD prophylaxis and biopsy, and were significantly at a higher risk of kidney failure (IS: 2/11, 18.1% vs. no IS: 2/6, 33.3%, = 0.04). "Associated extra-renal GvHD" occurred in 11/19 (57.9%) allogenic recipients. Patients with "associated extra-renal GvHD" had significantly more deaths (6/11, 60% vs. 0, = 0.02) but comparable renal outcomes.
Renal GvHD can present with or without "associated extra-renal GvHD" after a prolonged period of withdrawal of GvHD prophylaxis, requiring careful diagnostic vigilance and consideration of IS.
造血干细胞移植（HSCT）后，肾脏可能因多种原因受到损伤。其中，影响肾脏的移植物抗宿主病（GvHD）是一个未被充分认识的实体，在其诊断、临床病理表现和预后方面尚无明确指南。
在2005年至2020年间于本中心接受HSCT的2930例患者中，对19例同种异体和5例自体移植受者进行了肾脏活检。
该队列移植时的平均年龄为33.2±7岁，15例（62%）为男性。从HSCT到肾脏活检的中位时间为14（四分位间距，9 - 30）个月。再生障碍性贫血是最常见的潜在血液系统疾病（54.2%）。所有19例同种异体移植受者根据临床病理表现分为血栓性微血管病（TMA，12/19 [63%]）或肾病综合征（NS，7/19 [37%]）模式。肾小球毛细血管袢“系膜溶解”是12例TMA模式中9例所观察到的主要损伤模式。主要局限于肾小球区域的急性微血管病变占优势。在7例NS模式患者中，4例（57%）为膜性肾病，3例（43%）为微小病变肾病。39%（7/18）的患者C4d染色阳性，主要为肾小球阳性。未接受肾脏疾病免疫抑制（IS）的同种异体移植受者活检时的估算肾小球滤过率较低，从撤减GvHD预防措施到活检的间隔时间较长，且肾衰竭风险显著更高（IS组：2/11，18.1% vs. 无IS组：2/6，33.3%，P = 0.04）。“相关肾外GvHD”发生在11/19（57.9%）的同种异体移植受者中。有“相关肾外GvHD”的患者死亡显著更多（6/11，60% vs. 0，P = 0.02），但肾脏预后相当。
在撤减GvHD预防措施较长时间后，肾GvHD可能伴有或不伴有“相关肾外GvHD”，需要仔细的诊断警惕并考虑使用IS。
