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Histiocytosis, Langerhans-Cell (CUI C0019621) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0019621

NCI Thesaurus Code: C3107  (see NCI Thesaurus info)

Semantic Type: Neoplastic Process

NCIt Definition: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001)

RADLEX Definition: Group of disorders characterized by monoclonal proliferation of CD1+ histiocytes. The demonstration of the langerhans cells birbeck granules is the gold standard of the phenotype. eosinophilic granuloma makes up 60-80% of all the cases. [MeSH]

GARD Definition: Langerhans cell histiocytosis (LCH) is a disorder that primarily affects children, but is also found in adults of all ages. People with LCH produce too many Langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. In people with LCH, these cells multiply excessively and build up in certain areas of the body, causing tumors called granulomas to form. The symptoms vary among affected individuals, and the cause of LCH is unknown. In most cases, this condition is not life-threatening. Some people do experience life-long problems associated with LCH. - this information is from GARD/ORDR/NCATS.

NCI-GLOSS Definition: A group of rare disorders in which too many Langerhans cells (a type of white blood cell) grow in certain tissues and organs including the bones, skin, and lungs, and damage them. Langerhans cell histiocytosis may also affect the pituitary gland (which makes hormones that control other glands and many body functions, especially growth). Langerhans cell histiocytosis is most common in children and young adults.

NICHD Definition: Tissue infiltration by Langerhans cells (histiocytes). When infiltration involves the pituitary stalk or gland, presentation includes diabetes insipidus, which is sometimes accompanied by other pituitary dysfunction.

CTEP Definition: CTEP_For_FDA: 10025581/Malignant histiocytosis

CSP Definition: group of disorders of histiocyte proliferation which includes Letterer-Siwe disease; Hand-Schueller-Christian syndrome; and eosinophilic granuloma; Langerhans cells are components of the lesions.

MSH Definition: A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.

Synonyms & Abbreviations: (see Synonym Details)
Aleukemic Reticuloendothelioses, Systemic
Aleukemic Reticuloendotheliosis, Systemic
Cell Granulomatoses, Langerhans
Cell Granulomatosis, Langerhans
Cell Histiocytoses, Langerhans
Cell Histiocytosis, Langerhans
Chronic differentiated progressive histiocytosis
Chronic disseminated histiocytosis X
Chronic Histiocytosis X
Chronic idiopathic xanthomatosis
Differentiated progressive histiocytosis
Disease, Hand-Schueller-Christian
Disease, Hand-Schüller-Christian
Disease, Schueller-Christian
Eosinophilic granuloma (formerly)
EOSINOPHILIC GRANULOMA, MULTIFOCAL
Generalised histiocytosis of bones
Generalized Histiocytoses
Generalized histiocytosis of bones
Generalized Histiocytosis
Granulomatoses, Langerhans Cell
Granulomatosis, Langerhans Cell
Granulomatosis, Langerhans-Cell
Hand Schueller Christian Disease
Hand Schueller Christian Syndrome
Hand Schuller Christian disease
Hand Schüller Christian Disease
Hand Schüller Christian Syndrome
Hand-Schueller-Christian Disease
HAND-SCHUELLER-CHRISTIAN SYNDROME
Hand-Schuller-Christian disease [obs]
Hand-Schuller-Christian disease
Hand-Schüller-Christian syndrome (formerly)
Hand-Schüller-Christian disease (disorder)
Hand-Schüller-Christian disease
Hand-Schüller-Christian Syndrome
Histiocytoses, Generalized
Histiocytoses, Langerhans Cell
Histiocytoses, Type 2
Histiocytosis X [obs]
HISTIOCYTOSIS X II
Histiocytosis X syndrome (disorder)
Histiocytosis X syndrome
Histiocytosis X, NOS
histiocytosis X
Histiocytosis, Generalized
Histiocytosis, Langerhans Cell
Histiocytosis, Langerhans-Cell [Disease/Finding]
Histiocytosis, Langerhans-Cell
Histiocytosis, Type 2
Histiocytosis-X
Langerhan's cell histiocytosis
Langerhans cell disease
Langerhans Cell Granulomatoses
Langerhans cell granulomatosis
Langerhans Cell Histiocytoses
Langerhans cell histiocytosis (disorder)
Langerhans cell histiocytosis, multifocal (morphologic abnormality)
Langerhans cell histiocytosis, multifocal
Langerhans cell histiocytosis, no ICD-O subtype
Langerhans cell histiocytosis, no International Classification of Diseases for Oncology subtype (morphologic abnormality)
Langerhans cell histiocytosis, no International Classification of Diseases for Oncology subtype
Langerhans cell histiocytosis, NOS
Langerhans Cell Histiocytosis, Not Otherwise Specified
Langerhans cell histiocytosis, poly-ostotic
Langerhans cell histiocytosis, polyostotic
Langerhans cell histiocytosis
Langerhans' cell granulomatosis
Langerhans' cell histiocytosis
Langerhans-Cell Granulomatosis
Langerhans-cell histiocytosis NOS
Langerhans-Cell Histiocytosis
Langerhanszell-Histiozytose
LCH - Langerhan's cell histiocytosis
LCH
Letterer-Siwe Disease
Non Lipid Reticuloendotheliosis
Non-Lipid Reticuloendothelioses
Non-Lipid Reticuloendotheliosis
Reticuloendothelioses, Non-Lipid
Reticuloendothelioses, Systemic Aleukemic
Reticuloendotheliosis, Non-Lipid
Reticuloendotheliosis, Systemic Aleukemic
Schueller Christian Disease
SCHUELLER CHRISTIAN DIS
SCHUELLER-CHRISTIAN DISEASE
Schuller-Christian syndrome
Schüller-Christian syndrome
Syndrome, Hand-Schueller-Christian
Syndrome, Hand-Schüller-Christian
Systemic Aleukemic Reticuloendothelioses
Systemic Aleukemic Reticuloendotheliosis
Type 2 Histiocytoses
Type 2 Histiocytosis

External Source Codes: 
NCI Thesaurus Code C3107 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000020002 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
Contributing_Source CTEP NCI
Contributing_Source CTRP NCI
Contributing_Source NICHD NCI
CTV3ID C37y0 SNOMEDCT_US
CTV3ID C37y5 SNOMEDCT_US
CTV3ID XU7Gp SNOMEDCT_US
CTV3ID XU7Gr SNOMEDCT_US
CTV3ID XUILh SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_FIRST_PUBLISHED 2009-04-23 PDQ
DATE_LAST_MODIFIED 2009-04-23 PDQ
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000073002 SNOMEDCT_US
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 0427-5330 CSP
DISEASE_IDENTIFIER_ID 33010 GARD
DISEASE_IDENTIFIER_ID 4386 GARD
DISEASE_IDENTIFIER_ID 4387 GARD
DISEASE_IDENTIFIER_ID 4388 GARD
DISEASE_IDENTIFIER_ID 4390 GARD
DX 19760101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
HAS_GARD_PAGE true GARD
HN 90(75); was see under RETICULOENDOTHELIOSIS 1966-75; was HISTIOCYTOSIS X 1975-89 MSH
ICD-O-3_Code 9751/1 NCI
ICD-O-3_Code 9751/3 NCI
ICD-O-3_Code 9752/1 NCI
ICD-O-3_Code 9753/1 NCI
ICD-O-3_Code 9754/3 NCI
ICD-O-3_CODE 9751/3 SNOMEDCT_US
ICD-O-3_CODE 9753/1 SNOMEDCT_US
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
MDA 19750725 MSH
MESH_DEFINITION A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder. NDFRT
MESH_DUI D006646 NDFRT
MESH_NAME Histiocytosis, Langerhans-Cell NDFRT
MESH_UI M0010406 NDFRT
MIMTYPE 0 OMIM
MIMTYPEMEANING Other, mainly phenotypes with suspected mendelian basis OMIM
MIMTYPEVALUE none OMIM
MMR 20130708 MSH
MN C08.381.483.375 MSH
MN C15.604.250.400 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
NCI_THESAURUS_CODE C3107 PDQ
NDFRT_KIND DISEASE_KIND NDFRT
Neoplastic_Status Undetermined NCI
NICHD_Hierarchy_Term Langerhans Cell Histiocytosis NCI
NUI N0000001544 NDFRT
OBS [obs] ICDO
OL use HISTIOCYTOSIS, LANGERHANS-CELL to search HISTIOCYTOSIS X 1975-89; use RETICULOENDOTHELIOSIS 1966-74 MSH
ORIG_STY Neoplasm diagnosis PDQ
PM 90; was see under RETICULOENDOTHELIOSIS 1965-75; was HISTIOCYTOSIS X 1975-89 MSH
PRIMARY_PATH 10069698$10020118$10018865$10029104$Langerhans' cell histiocytosis$Histiocytoses$Haematopoietic neoplasms (excl leukaemias and lymphomas)$Neoplasms benign, malignant and unspecified (incl cysts and polyps) MDR
PRIMARY_SOC 10029104 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 20.0 MDR
PT_IN_VERSION 20.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/6858/langerhans-cell-histiocytosis/resources/1 GARD
RXAUI 3170967 RXNORM
RXAUI 3173137 RXNORM
RXAUI 3173139 RXNORM
RXAUI 3173141 RXNORM
RXAUI 3173142 RXNORM
RXAUI 3173152 RXNORM
RXAUI 3188144 RXNORM
RXAUI 3188145 RXNORM
RXAUI 3236806 RXNORM
RXAUI 3605875 RXNORM
RXAUI 3605876 RXNORM
RXAUI 3605877 RXNORM
RXAUI 4223510 RXNORM
RXAUI 4223854 RXNORM
RXAUI 4223976 RXNORM
RXAUI 5039567 RXNORM
RXAUI 5931345 RXNORM
RXCUI 989741 RXNORM
SMQ_TERM_ADDVERSION 12.0 MDR
SMQ_TERM_ADDVERSION 13.0 MDR
SMQ_TERM_ADDVERSION 9.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 12.0 MDR
SMQ_TERM_LMVERSION 13.0 MDR
SMQ_TERM_LMVERSION 20.0 MDR
SMQ_TERM_LMVERSION 9.0 MDR
SMQ_TERM_SCOPE 1 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 190955000 NDFRT
SNOMED_CID 190956004 NDFRT
SNOMED_CID 65399007 NDFRT
SUBSET_MEMBER 446608001~MAPTARGET~9751/3 SNOMEDCT_US
SUBSET_MEMBER 446608001~MAPTARGET~9753/1 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS C96.5 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS C96.6 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~C96.5 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~C96.6 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS C96.5 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS C96.6 | DESCENDANTS NOT EXHAUSTIVELY MAPPED SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS C96.6 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~C96.5 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~C96.6 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~C37y0 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~C37y5 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XU7Gp SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XU7Gr SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUILh SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERM_LEVEL 4 ICDO
TERM_LEVEL Related ICDO
TERM_LEVEL Synonym ICDO
TERMUI T018895 MSH
TERMUI T018896 MSH
TERMUI T020011 MSH
TERMUI T020012 MSH
TERMUI T020013 MSH
TERMUI T020014 MSH
TERMUI T023615 MSH
TERMUI T023616 MSH
TERMUI T705960 MSH
TERMUI T768737 MSH
TERMUI T768825 MSH
TERMUI T768826 MSH
TERMUI T782967 MSH
TERMUI T782968 MSH
TERMUI T782969 MSH
TERMUI T811619 MSH
TERMUI T841871 MSH
TH GHR (2014) MSH
TH NLM (1966) MSH
TH NLM (1976) MSH
TH NLM (1990) MSH
TH NLM (2009) MSH
TH NLM (2011) MSH
TH NLM (2012) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0019621

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