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

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).

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

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.