FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term hemophagocytic lymphohistiocytosis ID (Ontology) DOID:0050120 (Human Disease)
Definition A lymphatic system disease that is characterized by an expansion of the monocyte-macrophage population and intense hemophagocytosis. It can occur de novo, but more often occurs in the setting of another disorder, usually an infection or a malignancy. A clinical picture of fever, hepatosplenomegaly, lymphadenopathy and peripheral pancytopenia. The morphologic hallmark of this syndrome is the phagocytosis of hematopoietic elements by morphologically normal macrophages.
Also Known As "haemophagocytic syndrome"
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  immune system disease
   |__lymphatic system disease
       |__hemophagocytic lymphohistiocytosis  3 rec.
           |__familial hemophagocytic lymphohistiocytosis 1
           |__familial hemophagocytic lymphohistiocytosis 2
           |__familial hemophagocytic lymphohistiocytosis 3 1 rec.
           |__familial hemophagocytic lymphohistiocytosis 4 1 rec.
           |__familial hemophagocytic lymphohistiocytosis 5 1 rec.
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Is a lymphatic system disease
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Synonyms
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GARD:6589
ICD10CM:D76.1
MESH:D051359
MIM:PS267700
NCI:C34792
ORDO:540
SNOMEDCT_US_2023_03_01:190958003
UMLS_CUI:C0024291