Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Hemophagocytic lymphohistiocytosis classification
Introduction
Overview
Overview
Classification
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
References
References
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]