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Case Reports
. 2024 Oct 16:15:1464616.
doi: 10.3389/fimmu.2024.1464616. eCollection 2024.

Case report: Nephrotic syndrome and portal hypertensive ascites after allogeneic hematopoietic stem cell transplantation: a rare manifestation of chronic graft-versus-host disease

SanXi Ai #  1 , YuBing Wen #  1 , XiaoHong Fan  1 , TianRui Hua  2 , Wei Ye  1 , XueMei Li  1 , Yan Qin  1
Affiliations
Case Reports

Case report: Nephrotic syndrome and portal hypertensive ascites after allogeneic hematopoietic stem cell transplantation: a rare manifestation of chronic graft-versus-host disease

SanXi Ai et al. Front Immunol. .

Abstract

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.

Keywords: ascites; chronic GVHD; membranous nephropathy (MN); ruxolitinib; thrombotic microangiopathy (TMA).

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Light microscopic findings of glomeruli. (A) Endothelial swelling and subendothelial widening, Periodic acid-schiff stain, × 400. (B) Double contour of the glomerular basement membrane, Periodic acid-silver methenamine (PASM) stain, × 200. (C) Subendothelial exudation, PASM stain, × 400. (D) Microaneurysm, PASM stain, × 200. (E) Mesangiolysis, PASM stain, × 200. (F) Segmental spikes, PASM stain, × 400.
Figure 2
Figure 2
Immunofluorescence stainings and electron microscopic findings. (A) Fine granular staining for IgG along the glomerular tuft, × 200. (B) C4d staining along the glomerular tuft, × 200. (C) Endothelial swelling with subendothelial widening, × 4000. (D) Subepithelial electron-dense deposits, × 4000.
See this image and copyright information in PMC

References

    1. Jagasia MH, Greinix HT, Arora M, Williams KM, Wolff D, Cowen EW, et al. National Institutes of Health Consensus Development Project on criteria for clinical trials in chronic graft-versus-host disease: I. The 2014 diagnosis and staging working group report. Biol Blood Marrow Transplant. (2015) 21:389–401.e1. doi: 10.1016/j.bbmt.2014.12.001 - DOI - PMC - PubMed
    1. Cuvelier GDE, Schoettler M, Buxbaum NP, Pinal-Fernandez I, Schmalzing M, Distler JHW, et al. Toward a better understanding of the atypical features of chronic graft-versus-host disease: A report from the 2020 National Institutes of Health Consensus Project Task Force. Transplant Cell Ther. (2022) 28:426–45. doi: 10.1016/j.jtct.2022.05.038 - DOI - PMC - PubMed
    1. Hingorani S. Renal complications of hematopoietic-cell transplantation. N Engl J Med. (2016) 374:2256–67. doi: 10.1056/NEJMra1404711 - DOI - PubMed
    1. Brukamp K, Doyle AM, Bloom RD, Bunin N, Tomaszewski JE, Cizman B. Nephrotic syndrome after hematopoietic cell transplantation: do glomerular lesions represent renal graft-versus-host disease? Clin J Am Soc Nephrol. (2006) 1:685–94. doi: 10.2215/CJN.00380705 - DOI - PubMed
    1. Sethi S, Madden B, Casal Moura M, Nasr SH, Klomjit N, Gross L, et al. Hematopoietic stem cell transplant-membranous nephropathy is associated with protocadherin FAT1. J Am Soc Nephrol. (2022) 33:1033–44. doi: 10.1681/ASN.2021111488 - DOI - PMC - PubMed

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