造血干细胞移植受者的肾功能障碍模式和肾活检概况。

Glomerular diseases Pub Date : 2023-03-14 eCollection Date: 2023-01-01 DOI:10.1159/000529699
Elenjickal Elias John, Sanjeet Roy, Anup J Devasia, Reka Karuppusami, Nisha Jose, Selvin Sundar Raj Mani, Jeethu Joseph Eapen, Sabina Yusuf, Athul Thomas, Anna T Valson, Vinoi George David, Vikram Mathews, Biju George, Santosh Varughese, Suceena Alexander
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引用次数: 0

摘要

简介造血干细胞移植(HSCT)后,肾脏可能受到各种原因的损伤。其中,影响肾脏的移植物抗宿主疾病(GvHD)尚未得到充分认识,在其诊断、临床病理表现和预后方面没有明确的指南:2005年至2020年期间,在本中心接受造血干细胞移植的2930名患者中,有19名异体受者和5名自体受者接受了肾活检:结果:患者移植时的平均年龄为(33.2 ± 7)岁,其中 15 例(62%)为男性。从造血干细胞移植到肾活检的中位时间为14个月(IQR,9-30个月)。再生障碍性贫血是最常见的基础血液病(54.2%)。根据临床病理表现,所有 19 例异基因受者均被分为血栓性微血管病(TMA,12/19 [63%])或肾病综合征(NS,7/19 [37%])两种模式。在 9/12 例 TMA 型病例中,肾小球簇 "系膜溶解 "是主要的损伤模式。急性微血管病变主要局限于肾小球区。在 7 例 NS 型患者中,4 例(57%)出现膜性肾病,3 例(43%)出现微小病变。39%的患者(7/18)C4d染色呈阳性,主要是肾小球。未因肾脏疾病接受免疫抑制(IS)的异体受者活检时的eGFR较低,从停止预防GvHD到活检之间的潜伏期较长,肾衰竭的风险明显较高(IS:2/11,18.1% vs. 无IS:2/6,33.3%,p = 0.04)。11/19(57.9%)名异基因受者出现了 "相关肾外GvHD"。伴有肾外GvHD "的患者死亡人数明显增多(6/11,60% vs. 0,p = 0.02),但肾脏结果相当:结论:长期停用预防性肾脏坏死药物后,肾脏坏死可伴有或不伴有 "相关肾外坏死",因此需要在诊断时保持警惕并考虑IS。
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Patterns of Renal Dysfunction and Profile of Kidney Biopsies in Hematopoietic Stem Cell Transplant Recipients.

Introduction: 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.

Material and methods: 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.

Results: 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%, p = 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, p = 0.02) but comparable renal outcomes.

Conclusion: 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.

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