Hemophagocytic Syndrome

Preferred Label : Hemophagocytic Syndrome;

Obsolete resource : true;

NCIt definition : A classification of rare, non-neoplastic, proliferative disorders of the hematologic system. Primary or familial hemophagocytic lymphohistiocytosis (HLH) is inherited with an autosomal recessive pattern with five known subtypes differentiated by specific genetic mutations. Secondary manifestation of HLH is usually seen in hyperactivated immunologic states such as infection, autoimmune disease or malignancy. Histiocytes in the bone marrow, spleen or lymph nodes become activated to the point that phagocytosis proceeds unchecked. Clinical signs usually present within the first decade and include fever, jaundice, hepatosplenomegaly, lymphadenopathy and skin rash. Prompt initiation of treatment improves survival though prognosis remains poor even with intervention.;

NCIt note : See 'Familial Hemophagocytic Lymphohistiocytosis(C61276)';

Concept status : Retired_Concept;

Details

Origin ID

C35439;

UMLS CUI

C3887558;

Currated CISMeF NLP mapping
- Haemophagocytic lymphohistiocytosis [ICD-10 Sub-category (who)]
- Haemophagocytic lymphohistiocytosis [MedDRA Preferred Term]
- Haemophagocytic lymphohistiocytosis [MedDRA LLT]
- Haemophagocytic syndrome [MedDRA LLT]
- Hemophagocytic Syndromes [MeSH concept]
- Hemophagocytic lymphohistiocytosis [ICD-10 Sub-category]
- Hemophagocytic lymphohistiocytosis [MedDRA LLT]
- Hemophagocytic lymphohistiocytosis [PASCAL descriptor]
- Hemophagocytic lymphohistiocytosis (disorder) [SNOMED CT concept]
- Hemophagocytic syndrome [PASCAL descriptor]
- Hemophagocytic syndrome [ORDO Disease]
- Hemophagocytic syndrome [MedDRA LLT]
- Hemophagocytic syndrome (disorder) [Inactive SNOMED CT concept]
- Hemophagocytic syndromes [ICD-9 code]
- 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]
Disease may have findings
- Pancytopenia [NCIt concept]
See also inter- (CISMeF)
- Primary haemophagocytic lymphohistiocytosis [ICD-11 Sub-sub category]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Haemophagocytic syndrome [MedDRA LLT]
- Hemophagocytic syndrome [MedDRA LLT]
- Hemophagocytic syndrome (disorder) [Inactive SNOMED CT concept]
- Hemophagocytic syndromes [ICD-9 code]
disease_has_finding
- Hemophagocytosis [NCIt concept]

Keyword's position in hierarchy(ies) :

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