Table 5.
Histiocytoses of the H group (HLH)
| Histiocytoses of the H group |
|---|
| Primary HLH: Mendelian inherited conditions leading to HLH |
| HLH associated with lymphocyte cytotoxic defects |
| FHL2 (PRF1) |
| FHL3 (UNC13D) |
| FHL4 (STX11) |
| FHL5 (STXBP2) |
| XLP1 (SH2D1A) |
| Griscelli Syndrome type 2 (RAB27A) |
| Chediak-Higashi Syndrome (LYST) |
| HLH associated with abnormalities of inflammasome activation |
| XLP2 (BIRC4) |
| NLRC4 |
| HLH associated with defined Mendelian disorders affecting inflammation |
| Lysinuric protein intolerance (SLC7A7) |
| HMOX1 |
| Other defined Mendelian disorders affecting inflammation |
| Familial (apparently Mendelian) HLH of unknown origin |
| Secondary HLH (apparently non-Mendelian HLH) |
| Infection-associated HLH |
| Virus-associated HLH |
| EBV-associated HLH |
| CMV-associated HLH |
| HLH associated with other defined herpes virus infections |
| HIV-associated HLH |
| Influenza-associated HLH |
| HLH associated with other defined virus infections |
| Bacteria-associated HLH* |
| Parasite-associated HLH* |
| Fungal-associated HLH* |
| Malignancy-associated HLH |
| Malignancy-triggered HLH (HLH at onset of malignancy) |
| Hematological malignancies |
| T-cell lymphoblastic lymphoma/leukemia |
| T-cell non-lymphoblastic lymphomas |
| B-cell leukemias |
| B-cell lymphomas (non-Hodgkin) |
| Hodgkin lymphomas |
| NK-cell lymphomas/leukemias |
| Myeloid neoplasia |
| Other hematological malignancies |
| Solid tumors |
| Unclassified malignancies |
| HLH occurring during chemotherapy (not associated with initial diagnosis of malignancy)* |
| HLH associated with a malignancy, but not further defined |
| HLH associated with defined rheumatologic conditions (MAS-HLH, or MAS-HLH) |
| HLH associated with SoJIA |
| HLH associated with adult-onset Still disease |
| HLH associated with SLE |
| HLH associated with vasculitis |
| HLH associated with other defined autoimmune conditions |
| HLH associated with a not defined autoimmune condition |
| Transplant-related HLH* |
| HLH associated with iatrogenic immune activation* |
| HLH associated with iatrogenic immune suppression |
| HLH associated with other apparently non-Mendelian conditions |
| HLH of unknown/uncertain origin |
CMV, cytomegalovirus; EBV, Epstein-Barr virus; NK, natural killer; SoJIA, systemic-onset juvenile idiopathic arthritis.
*
See supplemental Table 1 for detailed list.