Preferred Name

hemophagocytic lymphohistiocytosis

Synonyms

haemophagocytic syndrome

Definitions

Xref MGI. MESH:C537250 added from NeuroDevNet [WAK]. 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.

ID

http://purl.obolibrary.org/obo/DOID_0050120

comment

Xref MGI. MESH:C537250 added from NeuroDevNet [WAK].

database_cross_reference

MESH:D051359

SNOMEDCT_US_2023_03_01:190958003

ICD10CM:D76.1

OMIM:PS267700

UMLS_CUI:C0024291

GARD:6589

NCI:C34792

ORDO:540

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.

has exact synonym

haemophagocytic syndrome

has symptom

http://purl.obolibrary.org/obo/SYMP_0019142

http://purl.obolibrary.org/obo/SYMP_0000521

http://purl.obolibrary.org/obo/SYMP_0000047

has_alternative_id

DOID:6453

has_obo_namespace

disease_ontology

id

DOID:0050120

in_subset

http://purl.obolibrary.org/obo/doid#NCIthesaurus

http://purl.obolibrary.org/obo/doid#DO_rare_slim

label

hemophagocytic lymphohistiocytosis

notation

DOID:0050120

prefLabel

hemophagocytic lymphohistiocytosis

subClassOf

http://purl.obolibrary.org/obo/DOID_75

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Delete Mapping To Ontology Source
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http://purl.bioontology.org/ontology/MEDDRA/10071580 MEDDRA LOOM
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http://purl.obolibrary.org/obo/DERMO_0003015 DERMO LOOM