Classification and underlying conditions of hemophagocytic lymphohistiocytosis (HLH).
| * Familial HLH was first described by Farquhar and Claireaux in 1952 |
| Genetic HLH |
| Familial HLH (Farquhar disease*) |
| Known gene defects (perforin, munc 13-4, syntaxin 11) |
| Unknown gene defects |
| Immune deficiency syndromes |
| Chédiak-Higashi syndrome (CHS) |
| Griscelli syndrome (GS) |
| X-linked lymphoproliferative syndrome (XLP) |
| Acquired HLH |
| Exogenous agents (infectious organisms, toxins) |
| Infection-associated hemophagocytic syndrome (IAHS) |
| Endogenous products (tissue damage, metabolic products) |
| Rheumatic diseases |
| Macrophage activation syndrome (MAS) |
| Malignant diseases |
| * Familial HLH was first described by Farquhar and Claireaux in 1952 |
| Genetic HLH |
| Familial HLH (Farquhar disease*) |
| Known gene defects (perforin, munc 13-4, syntaxin 11) |
| Unknown gene defects |
| Immune deficiency syndromes |
| Chédiak-Higashi syndrome (CHS) |
| Griscelli syndrome (GS) |
| X-linked lymphoproliferative syndrome (XLP) |
| Acquired HLH |
| Exogenous agents (infectious organisms, toxins) |
| Infection-associated hemophagocytic syndrome (IAHS) |
| Endogenous products (tissue damage, metabolic products) |
| Rheumatic diseases |
| Macrophage activation syndrome (MAS) |
| Malignant diseases |