Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2021.243
Autopsy and Case Reports
Autopsy Case Report

An autopsy series of an oft-missed ante-mortem diagnosis: hemophagocytic lymphohistiocytosis

Anusree Majumder; Debraj Sen

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal syndrome resulting from a hyperactivated immune system. Diverse patient profiles and clinical presentations often result in misdiagnosis. This article describes the varied clinical presentations and autopsy findings in three patients with this entity. The etiopathogenesis of HLH, its disparate and confounding clinical features, the diagnostic criteria, and management principles are also briefly reviewed.

Keywords

Autopsy, Lymphohistiocytosis, Hemophagocytic, Macrophage Activation Syndrome, Ferritins, Hypertriglyceridemia

References

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Submitted date:
08/17/2020

Accepted date:
12/09/2020

Publication date:
04/15/2021

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