Hemophagocytic lymphohistiocytosis classification

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Differentiating Hemophagocytic lymphohistiocytosis from other Diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Classification

HLH primarily categorized as

  • Familial (primary) hemophagocytic lymphohistiocytosis (FHL)
  • Secondary HLH (SHLH)

Familial forms

FHL, an autosomal recessive disorder, is invariably fatal when untreated. It is associated with defective triggering of apoptosis and reduced cytotoxic activity, resulting in a widespread accumulation of T lymphocytes and activated macrophages.

There are four types, and each is associated with a specific gene:

Familial hemophagocytic lymphohistiocytosis has an autosomal recessive pattern of inheritance.


References


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