hemophagocytic syndrome
Hemophagocytic syndrome (HPS) is a rare immune disease and a potentially life-threatening disorder characterized by cytokine storm and overwhelming inflammation causing fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. It can be either primary due to a genetic defect (primary hemophagocytic lymphohistiocytosis), or secondary to malignancies, to infections, most commonly with viruses such as Epstein-Barr virus or cytomegalovirus, human immunodeficiency virus, or to autoimmune disorders such as systemic lupus erythematosus or adult-onset Still disease (secondary hemophagocytic lymphohistiocytosis).
MONDO:0002254
syndromic disease
is_a
MONDO:0005833
lymphatic system disorder
is_a
FHL
related
HLH
exact
Hemophagocytic Lymphohistiocytosis
exact
familial erythrophagocytic lymphohistiocytosis
related
familial hemophagocytic lymphohistiocytosis
related
familial histiocytic reticulosis
related
hemophagocytic lymphohistiocytosis
exact
hemophagocytic syndrome
exact
SPCC825.03c SPAC23H3.06 SPAC11E3.02c SPBC28E12.06c SPCC584.05