Hematology · Hematology / Histiocytic disorders
Hemophagocytic lymphohistiocytosis
also: hemophagocytic syndrome; HLH
Hyperinflammatory syndrome from uncontrolled activation of T cells and macrophages.
- Specialty
- Hematology
- Subspecialty
- Hematology / Histiocytic disorders
- Sex & age distribution
- Male: affected; Female: affected; Children: yes, primary HLH in infancy
- Gene(s) involved
- AP3B1, CD27, ITK, LYST, MAGT1, PRF1, RAB27A, SH2D1A, STX11, STXBP2, UNC13D, XIAP
- Alternative names & eponyms
- hemophagocytic syndrome; HLH
Etiology & mechanism
Defective cytotoxic killing leads to cytokine storm.
Gene(s) involved (12)
Symptoms & signs (8)
In plain terms, someone with Hemophagocytic lymphohistiocytosis may experience the following — click any to explore related syndromes:
Same gene(s)
Other syndromes that involve one of this syndrome's genes.
Overlapping symptoms
Other syndromes that share symptoms with this one.
Drag nodes, scroll to zoom, click a node to open that syndrome.
Shares symptoms with
1 shared
Alström syndrome
hypertriglyceridemia
1 shared
Atypical hemolytic uremic syndrome
neurologic involvement
1 shared
Autoimmune lymphoproliferative syndrome
splenomegaly
1 shared
Autoimmune polyglandular syndrome type 1
hepatitis
1 shared
Castleman syndrome
cytopenias
1 shared
Congenital generalized lipodystrophy
hypertriglyceridemia
1 shared
Deficiency of adenosine deaminase 2 syndrome
cytopenias
1 shared
DRESS syndrome
hepatitis
1 shared
Evans syndrome
splenomegaly
1 shared
Familial partial lipodystrophy
hypertriglyceridemia
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