Hemophagocytic Lymphohistiocytosis

Preferred Label : Hemophagocytic Lymphohistiocytosis;

NCIt synonyms : Hemophagocytic Disorder;

NCIt related terms : HLH;

NCIt definition : A rare, but potentially life-threatening disorder, characterized by the proliferation of histiocytes and macrophages, and phagocytosis of red blood cells, white blood cells, and platelets. It may be inherited or secondary due to infections, autoimmune disorders, or underlying malignancies. Signs and symptoms include fever, lymphadenopathy, hepatomegaly, splenomegaly, and pancytopenia.;

Alternative definition : NCI-GLOSS: A rare disorder in which histiocytes and lymphocytes (types of white blood cells) build up in organs including the skin, spleen, and liver, and destroy other blood cells. Hemophagocytic lymphohistiocytosis may be inherited or caused by certain conditions or diseases, including infections, immunodeficiency (inability of the body to fight infections), and cancer.;

Codes from synonyms : CDR0000523322; CDR0000488415;

Details

Origin ID

C34792;

UMLS CUI

C0024291;

CISMeF manual mappings
- Hemophagocytic syndrome [ORDO Disease]
Currated CISMeF NLP mapping
- Haemophagocytic lymphohistiocytosis [MedDRA LLT]
- Haemophagocytic lymphohistiocytosis [MedDRA Preferred Term]
- Haemophagocytic lymphohistiocytosis [ICD-10 Sub-category (who)]
- Haemophagocytic lymphohistiocytosis (disorder) [Inactive SNOMED CT concept]
- Haemophagocytic syndrome [MedDRA LLT]
- Hemophagocytic Syndromes [MeSH concept]
- Hemophagocytic lymphohistiocytosis [MedDRA LLT]
- Hemophagocytic lymphohistiocytosis [PASCAL descriptor]
- Hemophagocytic lymphohistiocytosis [ICD-10 Sub-category]
- Hemophagocytic lymphohistiocytosis (disorder) [SNOMED CT concept]
- Hemophagocytic syndrome [MedDRA LLT]
- Hemophagocytic syndrome [PASCAL descriptor]
- Hemophagocytic syndrome (disorder) [Inactive SNOMED CT concept]
- Hemophagocytic syndromes [ICD-9 code]
- Primary haemophagocytic lymphohistiocytosis [ICD-11 Sub-sub category]
- haemophagocytic lymphohistiocytosis [COVID-19 Thesaurus concept]
- hemophagocytic lymphohistiocytosis [Radlex concept]
- hemophagocytic lymphohistiocytosis [DO Concept]
- hemophagocytic lymphohistiocytosis [Disease (Nov.)]
- lymphohistiocytosis, hemophagocytic [MeSH Descriptor]
- lymphohistiocytosis, hemophagocytic [MeSH concept]
- non-Langerhans cell histiocytosis [Radlex concept]
DO Cross reference
- hemophagocytic lymphohistiocytosis [DO Concept]
Disease may have findings
- Fever [NCIt concept]
- Hepatomegaly [NCIt concept]
- Lymphadenopathy [NCIt concept]
- Pancytopenia [NCIt concept]
- Splenomegaly [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Haemophagocytic lymphohistiocytosis [MedDRA LLT]
- Haemophagocytic lymphohistiocytosis [MedDRA Preferred Term]
- Haemophagocytic lymphohistiocytosis [ICD-10 Sub-category (who)]
- Hemophagocytic lymphohistiocytosis [MedDRA LLT]
- Hemophagocytic lymphohistiocytosis [ICD-10 Sub-category]
- Hemophagocytic lymphohistiocytosis [PASCAL descriptor]
- Hemophagocytic lymphohistiocytosis (disorder) [SNOMED CT concept]
- Hemophagocytic syndrome [ORDO Disease]
- hemophagocytic lymphohistiocytosis [Radlex concept]
- hemophagocytic lymphohistiocytosis [DO Concept]
- lymphohistiocytosis, hemophagocytic [MeSH concept]
- lymphohistiocytosis, hemophagocytic [MeSH Descriptor]
concept_is_in_subset
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
disease_has_abnormal_cell
- Atypical Histiocyte [NCIt concept]
disease_has_finding
- Hemophagocytosis [NCIt concept]
- Histiocytosis [NCIt concept]
- Rare Lesion [NCIt concept]
disease_has_normal_cell_origin
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Histiocyte [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
may_be_associated_disease_of_disease
- Primary Cutaneous Gamma-Delta T-Cell Lymphoma [NCIt concept]
- Recurrent Primary Cutaneous Gamma-Delta T-Cell Lymphoma [NCIt concept]
- Refractory Primary Cutaneous Gamma-Delta T-Cell Lymphoma [NCIt concept]

Keyword's position in hierarchy(ies) :

You can consult :

recommendations
documents concerning education
documents for patients