噬血细胞性淋巴组织细胞病:肝脏病理学家的实践回顾

IF 0.1 Q4 PATHOLOGY
Yuqing Xiong, A. Del Portillo, Ladan Fazlollahi, H. Remotti, Michael J. Lee, S. Lagana
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引用次数: 0

摘要

噬血细胞淋巴组织细胞病(HLH)是一种死亡率高的罕见疾病。肝脏受累是常见的,大多数患者表现为急性肝炎。因此,肝活检往往是在设定怀疑HLH。一些研究描述了HLH累及肝脏的临床病理表现。组织学是高度可变的,正如所证明的噬血细胞症的发生率。本文综述了HLH的临床特点,特别强调其肝脏表现。然后我们描述了常见和不常见的组织学模式。最后,我们讨论了肝脏病理学家在考虑这一实体时所面临的困难,并就最严重的“痛点”提供了我们的意见。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Hemophagocytic Lymphohistiocytosis: A Practical Review for Liver Pathologists
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.”
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