Kidney Week

Abstract: TH-PO714

Collapsing Focal Segmental Glomerulosclerosis in a Patient with Hemophagocytic Lymphohistiocytosis

Session Information

• Glomerular Diseases: Case Reports
  November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

• Nunez, Belen A., Memorial Sloan Kettering Cancer Center, New York, New York, United States

Belen A. Nunez,

• Salvatore, Steven, Weill Cornell Medicine, New York, New York, United States

Steven Salvatore,

• Jaimes, Edgar A., Memorial Sloan Kettering Cancer Center, New York, New York, United States

Edgar A. Jaimes,

• Shaikh, Aisha, Memorial Sloan Kettering Cancer Center, New York, New York, United States

Aisha Shaikh,

Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a systemic inflammatory disorder caused by the activation and proliferation of nonmalignant macrophages. HLH-associated podocytopathy is very rare. Here, we report a case of HLH-associated collapsing focal segmental glomerulosclerosis (FSGS).

Case Description

A 46-year-old black man with plasma cell leukemia presented to the hospital with a fever and dyspnea. CT scan showed bibasilar lung consolidation and antibiotics were started. He had anemia (hemoglobin 5 g/dl), thrombocytopenia (platelets 10,000 K/µl) and high ferritin (129,379 ng/ml), lactate dehydrogenase (3707 U/L), triglycerides (294 mg/dl), and soluble IL2 Receptor (2145 pg/ml). Based on these findings, a diagnosis of HLH was made. On admission, urine analysis showed 30 mg/dL protein; on hospital day #5, the urine-spot-protein-creatinine ratio (UPCR) was 60 g/g; urine albumin-creatinine-ratio was 25 g/g, and UPEP/IF revealed a monoclonal band with an M-spike of 8.3 mg/dl. Serum creatinine (SCr) rose to 3.8 mg/dl. One gram of solumedrol was administered daily for three days, followed by oral prednisone 60 mg daily. Kidney biopsy showed collapsing FSGS with rare sub-epithelial humps and no evidence of paraprotein disease. Blood cultures and tests for HIV, parvovirus, CMV, EBV, and SARS-CoV-2 were negative. Genotyping for APOL-1 risk alleles is pending. Two weeks following steroid initiation, UPCR improved to 1.5 g/g, and SCr was 1.2 mg/dl.

Discussion

Here, we report a case of HLH-associated collapsing FSGS with sub-epithelial humps. No infectious cause was identified, but the presence of sub-epithelial humps suggests an infectious trigger for HLH. HLH is characterized by cytokine release, which induces podocyte injury. HLH-associated collapsing FSGS occurs in black individuals with APOL-1 mutation. In a large case series of HLH-associated glomerular diseases¹, the mortality rate was 64%, and kidney recovery was rare. The rapid improvement in proteinuria and AKI in collapsing FSGS, as observed in this patient, is very rare.
¹ Nephrotic syndrome associated with hemophagocytic syndrome. Kidney Int. 2006.