- Uncontrolled activation of T Cells, w/inappropriate release of cytokines and macrophage activation
- Rx directed at underlying cause
- Triggered by lymphoma, leukemia, autoimmunes dz, infxns→ ebv, enterovirus, gastroenteritis, other
- Also a rare familial form
- Fever that's unexplained
- Other symptoms related to the inciting process (e.g. infection, cancer, auto-immune d/o, etc.)
- Cytopenias in at least 2 cell lines (WBC, HgB, Plt)
- Hypertriglyceridemia ( > 265) and/or decreased fibrinogen (< 150)
- Ferritin > 500 (often > 5,000)
- Bone marrow w/hemophagocytosis