Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis.

ABSTRACT:

Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome produced by dysregulated activation of the immune system. Acute kidney injury (AKI) and proteinuria have been infrequently described in the setting of HLH, and investigations of underlying histopathologic changes in the kidney are limited.

Methods

To characterize kidney pathology in HLH, a retrospective review of 30 patients' clinical and laboratory data, and kidney tissue was performed (18 from autopsy, and 12 biopsied patients).

Results

HLH was associated with infection (83%), autoimmune disease (37%), and malignancy (20%), including 30% with concurrent autoimmune disease and infection. Nephrological presentations included subnephrotic range proteinuria (63%), AKI (63%), hematuria

SUBMITTER: Sekulic M 

PROVIDER: S-EPMC10851018 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature