Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with a high mortality. Liver involvement is common, with most patients demonstrating acute hepatitis. Therefore, liver biopsies are frequently obtained in the setting of suspected HLH. Several studies have described the clinicopathologic manifestations of liver involvement by HLH. The histology is highly variable, as are rates of demonstrated hemophagocytosis. This review summarizes the clinical characteristics of HLH, with particular emphasis on hepatic manifestations thereof. We then describe the histologic patterns that have been commonly and uncommonly reported. Finally, we address difficulties faced by liver pathologists when considering this entity and offer our opinions regarding the worst “pain points.”

Yuqing Xiong

Armando Del Portillo

Ladan Fazlollahi

Helen E. Remotti

Michael Lee

Stephen M. Lagana

AJSP Review and Reports

AJSP: Reviews and Reports

Hemophagocytic Lymphohistiocytosis: A Practical Review for Liver Pathologists

噬血细胞性淋巴组织细胞增多症：肝脏病理学家的实用综述

Hemophagocytic Lymphohistiocytosis: A Practical Review for Liver Pathologists

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Journal Info

Category	Quartile
PATHOLOGY	4