234437005: Hemophagocytic lymphohistiocytosis (disorder)

SNOMED CT Concept\Clinical finding (finding)\...

\Finding of blood, lymphatics and immune system\Disorder of cellular component of blood (disorder)\White blood cell disorder\Qualitative abnormality of granulocyte\Combined phagocytic defect\Haemophagocytic lymphohistiocytosis

\Disease\Disorder of immune function (disorder)\Immunodeficiency disorder\Primary immune deficiency disorder\Phagocytic cell defect\Phagocytic cell dysfunction (disorder)\Combined phagocytic defect\Haemophagocytic lymphohistiocytosis
\Disease\Disorder of immune function (disorder)\White blood cell disorder\Qualitative abnormality of granulocyte\Combined phagocytic defect\Haemophagocytic lymphohistiocytosis
\Disease\Disorder of hematopoietic cell proliferation (disorder)\Histiocytic syndrome\Haemophagocytic lymphohistiocytosis
\Disease\Disorder of cellular component of blood (disorder)\White blood cell disorder\Qualitative abnormality of granulocyte\Combined phagocytic defect\Haemophagocytic lymphohistiocytosis
\Disease\Disorder of body system\Disorder of immune structure (disorder)\Combined phagocytic defect\Haemophagocytic lymphohistiocytosis
\Disease\Disorder of body system\Disorder of hematopoietic structure (disorder)\Haemophagocytic lymphohistiocytosis

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jan 2002. Module: SNOMED CT core

Descriptions:

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
Haemophagocytic lymphohistiocytosis	Is a	anémie hémolytique auto-immune avec érythrophagocytose (Fc portion de l'auto-anticorps)	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Finding site	Leukocyte	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Finding site	Structure of immune system (body structure)	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Finding site	Hematopoietic system structure	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Finding site	Hematopoietic system structure	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Associated morphology	White blood cell abnormality	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Is a	Disorder of hematopoietic structure (disorder)	true	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Associated morphology	Histiocytic proliferation - category	false	Inferred relationship	Some	1
Haemophagocytic lymphohistiocytosis	Has definitional manifestation	Immune system finding	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Has definitional manifestation	White blood cell finding	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Finding site	Mononuclear phagocyte system structure (body structure)	false	Inferred relationship	Some	2
Haemophagocytic lymphohistiocytosis	Has definitional manifestation	Immune system finding	false	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Pathological process (attribute)	Abnormal immune process (qualifier value)	false	Inferred relationship	Some	3
Haemophagocytic lymphohistiocytosis	Pathological process (attribute)	Abnormal immune process (qualifier value)	true	Inferred relationship	Some	1
Haemophagocytic lymphohistiocytosis	Finding site	Mononuclear phagocyte system structure (body structure)	true	Inferred relationship	Some	1
Haemophagocytic lymphohistiocytosis	Associated morphology	Histiocytic proliferation (morphologic abnormality)	true	Inferred relationship	Some	1
Haemophagocytic lymphohistiocytosis	Is a	Histiocytic syndrome	true	Inferred relationship	Some
Haemophagocytic lymphohistiocytosis	Is a	Combined phagocytic defect	true	Inferred relationship	Some

Inbound Relationships	Type	Active	Source	Characteristic	Refinability	Group
Primary hemophagocytic lymphohistiocytosis	Is a	False	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
Macrophage activation syndrome (disorder)	Is a	True	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
Hemophagocytic lymphohistiocytosis due to infection (disorder)	Is a	False	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
Hemophagocytic syndrome with human immunodeficiency virus infection (disorder)	Is a	True	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
Familial hemophagocytic lymphohistiocytosis (disorder)	Is a	True	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
A rare, secondary hemophagocytic lymphohistiocytosis characterized by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are leukemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings.	Is a	False	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
Hemophagocytic lymphohistiocytosis due to malignant disease	Is a	False	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
Secondary hemophagocytic lymphohistiocytosis (disorder)	Is a	True	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some
A rare unclassified autoinflammatory syndrome characterised by neonatal onset pancytopenia, type I interferon-dependent multisystemic autoinflammation, painful rash with variable frequencies and haemophagocytic lymphohistiocytosis. Failure to thrive, fever, gastrointestinal/upper respiratory tract infections, enterocolitis, hepatosplenomegaly, myelofibrosis and neurodevelopmental delay are other common clinical features. Facial dysmorphism including macrocephaly, mild frontal bossing, sparse hair, mild hypertelorism, depressed nasal bridge can be present.	Is a	True	Haemophagocytic lymphohistiocytosis	Inferred relationship	Some

This concept is not in any reference sets

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Id	Description	Lang	Type	Status	Case?	Module
351251017	Haemophagocytic lymphohistiocytosis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
351252012	Hemophagocytic lymphohistiocytosis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
622755016	Hemophagocytic lymphohistiocytosis (disorder)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
3701425010	Haemophagocytic syndrome	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
3701426011	Hemophagocytic syndrome	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
4589315014	Hyperinflammatory lymphohistiocytosis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
50009251000188110	lymphohistiocytose hémophagocytaire	fr	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
50009261000188112	syndrome hémophagocytaire	fr	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
50009271000188115	LHH	fr	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
2321391000195110	Hämophagozytäre Lymphohistiozytose	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
2321401000195113	Hämophagozytisches Syndrom	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
2321411000195110	Hyperinflammatorische Lymphohistiozytose	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
2321421000195117	HLH - hämophagozytische Lymphohistiozytose	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module
3408781001000117	Hämophagozytose-Syndrom	de	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Switzerland NRC maintained Module