title: Direct Reversible Kidney Injury in Familial Hemophagocytic Lymphohistiocytosis Type 3.
abstract: Hemophagocytic lymphohistiocytosis is a hyperinflammatory disorder resulting from primary or secondary immune dysfunction. AKI is frequent in severe hemophagocytic lymphohistiocytosis and has been attributed to multiorgan failure or the use of nephrotoxic drugs, but AKI is rarely considered a direct consequence of the disease process. We describe a child with familial hemophagocytic lymphohistiocytosis type 3 who developed AKI requiring prolonged renal replacement therapy because of severe renal inflammation. There was massive infiltration of the renal parenchyma by activated macrophages and cytotoxic T cells, and acute tubular injury. The patient responded to high-dose intravenous methylprednisolone, which resulted in improvement of renal function and discontinuation of renal replacement therapy. This case confirms the occurrence of reversible AKI due to hemophagocytic lymphohistiocytosis-induced activated macrophage infiltration of the renal parenchyma and inflammation.
authors: Department of Pediatrics, Divisions of Nephrology and.
authors: Laura
authors: L
authors: Malaga-Dieguez
authors: Pathology, New York University School of Medicine, New York, New York.
authors: Wu
authors: W
authors: Ming
authors: Department of Pediatrics, Divisions of Nephrology and howard.trachtman@nyumc.org.
authors: Howard
authors: H
authors: Trachtman
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D002648:Child; D006801:Humans; D007668:Kidney / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D008297:Male
keywords: acute renal failure; clinical nephrology; immunology and pathology