Hemophagocytic lymphohistiocytosis classification
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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:
- FHL1 - HPLH1
- FHL2 - PRF1
- FHL3 - UNC13D
- FHL4 - STX11
- FHL5 – STXBP2 (Syntaxin binding protein 2)/UNC18-2
