Characteristics of patients with IDCH
| . | Entire cohort (N = 43) . | Primary IDCH (n = 25, 58%) . | Secondary IDCH (n = 18, 42%) . | |
|---|---|---|---|---|
| Median age (y) | 70 | 64 | 77 | |
| Pediatric patients (n) | 7 | 6 | 1 | |
| Sex | ||||
| Female | 13 | 9 | 4 | |
| Male | 30 | 16 | 14 | |
| Location (n) | ||||
| Skin only | 28 | 20 | 8 | |
| Lymph node only | 8 | 2 | 6 | |
| Skin and lymph node | 3 | 1 | 2 | |
| Colonic polyp | 2 | 1 | 1 | |
| Tonsil | 1 | 0 | 1 | |
| Lung | 1 | 1 | 0 | |
| Immunohistochemistry (n) | ||||
| CD1a expression | 43/43 | 25/25 | 18/18 | |
| S100 protein expression | 31/40 | 16/22 | 15/18 | |
| CSF1R/CD115 expression | 7/29 | 4/16 | 3/13 | |
| CD1c expression | 16/17 | 9/9 | 7/8 | |
| Langerin expression: | ||||
| Langerin negative | 28 | 4/28 pediatric | 16 | 12 |
| 1/28 mixed histiocytosis | ||||
| with BRAF∗/MAP2K1: 5/28 | ||||
| Langerin positive (<20%) | 15 | 3/15 pediatric | 9 (36%) | 6 (33%) |
| 6/15 mixed histiocytosis | ||||
| with BRAF∗/MAP2K1: 9/15 | ||||
| Molecular alterations (n) | ||||
| KRAS | 13 | 4 | 9 | |
| BRAF∗ alterations | 13 | 8 | 5 | |
| ETV3::NCOA2 | 6 | 6 | 0 | |
| Mixed histiocytosis (n) | 7 | 4 | 3 | |
| with BRAF∗/MAP2K1: 7/7 | ||||
| Disease-related mortality | 12/34 (35%) | 2/20 (10%) | 10/14 (71%) | |
| . | Entire cohort (N = 43) . | Primary IDCH (n = 25, 58%) . | Secondary IDCH (n = 18, 42%) . | |
|---|---|---|---|---|
| Median age (y) | 70 | 64 | 77 | |
| Pediatric patients (n) | 7 | 6 | 1 | |
| Sex | ||||
| Female | 13 | 9 | 4 | |
| Male | 30 | 16 | 14 | |
| Location (n) | ||||
| Skin only | 28 | 20 | 8 | |
| Lymph node only | 8 | 2 | 6 | |
| Skin and lymph node | 3 | 1 | 2 | |
| Colonic polyp | 2 | 1 | 1 | |
| Tonsil | 1 | 0 | 1 | |
| Lung | 1 | 1 | 0 | |
| Immunohistochemistry (n) | ||||
| CD1a expression | 43/43 | 25/25 | 18/18 | |
| S100 protein expression | 31/40 | 16/22 | 15/18 | |
| CSF1R/CD115 expression | 7/29 | 4/16 | 3/13 | |
| CD1c expression | 16/17 | 9/9 | 7/8 | |
| Langerin expression: | ||||
| Langerin negative | 28 | 4/28 pediatric | 16 | 12 |
| 1/28 mixed histiocytosis | ||||
| with BRAF∗/MAP2K1: 5/28 | ||||
| Langerin positive (<20%) | 15 | 3/15 pediatric | 9 (36%) | 6 (33%) |
| 6/15 mixed histiocytosis | ||||
| with BRAF∗/MAP2K1: 9/15 | ||||
| Molecular alterations (n) | ||||
| KRAS | 13 | 4 | 9 | |
| BRAF∗ alterations | 13 | 8 | 5 | |
| ETV3::NCOA2 | 6 | 6 | 0 | |
| Mixed histiocytosis (n) | 7 | 4 | 3 | |
| with BRAF∗/MAP2K1: 7/7 | ||||
| Disease-related mortality | 12/34 (35%) | 2/20 (10%) | 10/14 (71%) | |
BRAF V600E mutation or BRAF fusions.