hemophagocytic lymphohistiocytosis

Term ID

DOID:0050120

Synonyms

haemophagocytic syndrome

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. (2)

References

GARD:6589
ICD10CM:D76.1
MESH:D051359
NCI:C34792
OMIM:PS267700
ORDO:540
SNOMEDCT_US_2023_03_01:190958003
UMLS_CUI:C0024291

Ontology

Human Disease ( DOID:0050120 )

Relationships

is a type of: lymphatic system disease

lymphatic system disease Show first 5 Terms
has subtype: familial hemophagocytic lymphohistiocytosis 1 familial hemophagocytic lymphohistiocytosis 2 familial hemophagocytic lymphohistiocytosis 3 familial hemophagocytic lymphohistiocytosis 4 familial hemophagocytic lymphohistiocytosis 5

familial hemophagocytic lymphohistiocytosis 1 familial hemophagocytic lymphohistiocytosis 2 familial hemophagocytic lymphohistiocytosis 3 familial hemophagocytic lymphohistiocytosis 4 familial hemophagocytic lymphohistiocytosis 5 Show first 5 Terms

Other Pages

Alliance (1)

Genes Involved

Zebrafish Models

Citations