DISEASES

Disease-gene associations mined from literature

Human genes for hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis [DOID:0050120]

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.

Synonyms:  hemophagocytic lymphohistiocytosis,  DOID:0050120,  HPS,  Haemophagocytic lymphohistiocytosis,  Hemophagocytic syndrome ...