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Hemophagocytic lymphohistiocytosis

MedGen UID:
6158
Concept ID:
C0024291
Disease or Syndrome
Synonyms: Hemophagocytic Lymphohistiocytoses; Hemophagocytic Lymphohistiocytosis; Lymphohistiocytoses, Hemophagocytic; Lymphohistiocytosis, Hemophagocytic
SNOMED CT: Hemophagocytic lymphohistiocytosis (234437005); Hemophagocytic syndrome (234437005); Hyperinflammatory lymphohistiocytosis (234437005)

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. [from NCI]

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