Terminology used in this review
| Term . | Abbreviation . | Use . |
|---|---|---|
| Hemophagocytic lymphohistiocytosis | HLH | General syndrome |
| Primary HLH | — | HLH driven by genetic inborn errors of immunity, which include HLH as a main feature of the disease, such as included in Table 4 |
| Secondary HLH | — | HLH predominantly driven by environmental/ acquired mechanisms (eg, infection, malignancy, rheumatic disease) |
| Familial HLH | FHL | HLH driven by genetic defects in PRF1, UNC13D, STX11, or STXBP2 resulting in profoundly impaired NK-cell and CD8+ T-cell cytotoxic function |
| Macrophage activation syndrome | MAS | HLH occurring due to a rheumatic disease (usually systemic JIA) or autoinflammatory mutation, often associated with high IL-18 |
| Cytokine release syndrome | CRS | HLH due to CAR T-cell or BiTE therapy |
| Term . | Abbreviation . | Use . |
|---|---|---|
| Hemophagocytic lymphohistiocytosis | HLH | General syndrome |
| Primary HLH | — | HLH driven by genetic inborn errors of immunity, which include HLH as a main feature of the disease, such as included in Table 4 |
| Secondary HLH | — | HLH predominantly driven by environmental/ acquired mechanisms (eg, infection, malignancy, rheumatic disease) |
| Familial HLH | FHL | HLH driven by genetic defects in PRF1, UNC13D, STX11, or STXBP2 resulting in profoundly impaired NK-cell and CD8+ T-cell cytotoxic function |
| Macrophage activation syndrome | MAS | HLH occurring due to a rheumatic disease (usually systemic JIA) or autoinflammatory mutation, often associated with high IL-18 |
| Cytokine release syndrome | CRS | HLH due to CAR T-cell or BiTE therapy |
BiTE, bispecific T-cell engager; CAR, chimeric antigen receptor; JIA, juvenile idiopathic arthritis.