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- B08fbd0f3c253d74b43a47aa85ee529c5 hasDbXref "Orphanet:158032" @default.
- B08fbd0f3c253d74b43a47aa85ee529c5 type Axiom @default.
- B08fbd0f3c253d74b43a47aa85ee529c5 annotatedProperty IAO_0000115 @default.
- B08fbd0f3c253d74b43a47aa85ee529c5 annotatedSource MONDO_0015540 @default.
- B08fbd0f3c253d74b43a47aa85ee529c5 annotatedTarget "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)." @default.