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Ontology Browser

Term:
Parent Terms Term With Siblings Child Terms
adult xanthogranuloma 
Erdheim-Chester disease 
hemophagocytic lymphohistiocytosis +   
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. (DO)
juvenile xanthogranuloma 
multicentric reticulohistiocytosis 
Niemann-Pick disease +   
reticulohistiocytic granuloma 
sea-blue histiocytosis  
Sinus Histiocytosis +   

Synonyms
Exact Synonyms: HPLH ;   HPS ;   haemophagocytic syndrome ;   hemophagocytic lymphohistiocytoses ;   hemophagocytic syndrome ;   hemophagocytic syndromes ;   infection-associated hemophagocytic syndrome ;   primary hemophagocytic hymphohistiocytosis ;   primary hemophagocytic lymphohistiocytosis ;   reactive hemophagocytic syndrome
Primary IDs: MESH:D051359
Xrefs: GARD:6589 ;   ICD10CM:D76.1 ;   MIM:PS267700 ;   NCI:C34792 ;   ORDO:540
Definition Sources: http://ghr.nlm.nih.gov/condition/familial-hemophagocytic-lymphohistiocytosis "DO" "DO", http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801997000500007&lng=pt&nrm=iso "DO" "DO"

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