Hemophagocytic Syndrome With Histiocytic Glomerulopathy and Intraglomerular Hemophagocytosis

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
Dominick SantorielloVivette D D'Agati

Abstract

Hemophagocytic syndrome (HPS), a rare and life-threatening disease, is characterized by hyperactivation of the immune system that causes hypercytokinemia and potential multiorgan failure. Acute kidney injury is the most common kidney manifestation of HPS and is generally considered a poor prognostic factor. Glomerular involvement is uncommon and usually manifests as either podocytopathy with collapsing glomerulopathy or thrombotic microangiopathy. We report a rare case of severe histiocytic glomerulopathy in a patient with HPS who presented with acute kidney injury and proteinuria. Kidney biopsy revealed massive glomerular infiltration by macrophages resembling proliferative glomerulonephritis accompanied by intraglomerular hemophagocytosis and mild features of glomerular thrombotic microangiopathy. The patient's kidney failure and proteinuria responded rapidly to high-dose pulse methylprednisolone followed by a tapering course of oral prednisone. Our case expands the renal pathologic spectrum of HPS to include histiocyte-rich glomerular infiltration and intraglomerular hemophagocytosis. Greater awareness of this entity is needed to ensure prompt recognition and appropriate therapy.

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Citations

Feb 27, 2018·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sidharth Kumar SethiShyam Bihari Bansal
Jan 18, 2019·Biomédica : revista del Instituto Nacional de Salud·María Adoración Martín-GómezMercedes Gómez-Morales
Jul 1, 2018·Journal of the American Society of Nephrology : JASN·Mark A Perazella, Anushree C Shirali
Dec 24, 2020·Internal Medicine Journal·Hassan IzzedineAlexis Mathian

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