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Sudden Unexpected Death due to Hemophagocytic Lymphohistiocytosis Syndrome
Author(s) -
Chute Dennis J.,
Rawley James,
Cox John,
Bready Robert J.
Publication year - 2013
Publication title -
journal of forensic sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.715
H-Index - 96
eISSN - 1556-4029
pISSN - 0022-1198
DOI - 10.1111/1556-4029.12131
Subject(s) - hemophagocytosis , hepatosplenomegaly , medicine , hemophagocytic lymphohistiocytosis , macrophage activation syndrome , autopsy , histiocytosis , immunology , pediatrics , sepsis , disease , pathology , bone marrow , pancytopenia
Abstract The hemophagocytic lymphohistiocytosis ( HLH ) syndrome is a hyperimmune disorder characterized by lymphohistiocytic infiltrations, elevated cytokine levels in the blood, macrophage activation, and hemophagocytosis, frequently presenting with a febrile septic picture. This unusual disease is more common in infancy and childhood than adulthood. It is classified as primary or familial when a genetic defect is identified and secondary or acquired when triggered by certain infections, autoimmune disorders, or malignancies. If or when such patients expire, they typically do so within a hospital or under a physician's care and so such cases rarely come to the attention of forensic pathologists. We report on the unexpected deaths of two hospitalized adult cases of HLH brought to autopsy without a premortem diagnosis. Postmortem examination demonstrated marked hepatosplenomegaly and lymphadenopathy in association with hemophagocytosis. Although very uncommon HLH must be considered in infants, children, or adults who die unexpectedly with an undiagnosed septic presentation.