Relevant clinical and biological features at progression of ISM patients that progressed to more advanced disease
| . | Patient ID . | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| #1 . | #7 . | #8 . | #10 . | #15 . | #22 . | #30 . | #34 . | #38 . | #41 . | #47 . | #102 . | #104 . | #108 . | |
| Age at onset/at progression, y | 35/60 | 76/78 | 24/64 | 0/31 | 63/76 | 73/76 | 33/61 | 25/37 | 64/69 | 30/64 | 28/46 | 50/51 | 48/49 | 31/62 |
| Sex | M | M | F | M | M | F | F | F | M | M | M | M | F | M |
| Disease subtype at progression | SM-AHNMD (NHL) | SM-AHNMD (MPN) | ASM | ASM | SSM | ASM | SSM | SSM | ASM | ASM | SSM | ASM | ASM | ASM |
| Organomegalies | Yes* | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes* | Yes* | Yes | No | Yes* | Yes* |
| Cytopenias | No | No | No | Yes | No | Yes | No | No | Yes | No | No | Yes | Yes | Yes |
| Bone lesions | O + F | O | O + F | O, DBS | No | DBS | DBS | DBS | O | No | DBS | O | DBS | DBS |
| Serum tryptase, ng/mL | 182 | 144 | 239 | 430 | 170 | 357 | 173 | 329 | 52.6 | 151 | 310 | 238 | 312 | 2036 |
| % of BM MCs | 0.4 | 0.6 | 9 | 11 | 3.2 | 18 | 0.06 | 0.35 | 0.13 | 0.5 | 3.1 | 0.47 | 3.7 | 4.8 |
| % of KIT D816V+ MSCs | 100 | 100 | 17 | 49 | 11 | 21 | — | 9 | — | — | 31 | 10 | 24 | 41 |
| KIT D816V+ BM hematopoietic cell compartments | MC + M + L | MC + M + L | MC + M + L | MC + M + L | MC + M + L | MC + M | MC + M | MC + M | MC + M | MC + M | MC + M | MC + M | MC + M + L | MC + M |
| . | Patient ID . | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| #1 . | #7 . | #8 . | #10 . | #15 . | #22 . | #30 . | #34 . | #38 . | #41 . | #47 . | #102 . | #104 . | #108 . | |
| Age at onset/at progression, y | 35/60 | 76/78 | 24/64 | 0/31 | 63/76 | 73/76 | 33/61 | 25/37 | 64/69 | 30/64 | 28/46 | 50/51 | 48/49 | 31/62 |
| Sex | M | M | F | M | M | F | F | F | M | M | M | M | F | M |
| Disease subtype at progression | SM-AHNMD (NHL) | SM-AHNMD (MPN) | ASM | ASM | SSM | ASM | SSM | SSM | ASM | ASM | SSM | ASM | ASM | ASM |
| Organomegalies | Yes* | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes* | Yes* | Yes | No | Yes* | Yes* |
| Cytopenias | No | No | No | Yes | No | Yes | No | No | Yes | No | No | Yes | Yes | Yes |
| Bone lesions | O + F | O | O + F | O, DBS | No | DBS | DBS | DBS | O | No | DBS | O | DBS | DBS |
| Serum tryptase, ng/mL | 182 | 144 | 239 | 430 | 170 | 357 | 173 | 329 | 52.6 | 151 | 310 | 238 | 312 | 2036 |
| % of BM MCs | 0.4 | 0.6 | 9 | 11 | 3.2 | 18 | 0.06 | 0.35 | 0.13 | 0.5 | 3.1 | 0.47 | 3.7 | 4.8 |
| % of KIT D816V+ MSCs | 100 | 100 | 17 | 49 | 11 | 21 | — | 9 | — | — | 31 | 10 | 24 | 41 |
| KIT D816V+ BM hematopoietic cell compartments | MC + M + L | MC + M + L | MC + M + L | MC + M + L | MC + M + L | MC + M | MC + M | MC + M | MC + M | MC + M | MC + M | MC + M | MC + M + L | MC + M |
DBS, diffuse bone sclerosis; ID, identification; MC + M, MC plus other myeloid lineage cells; MC + M + L, MC plus other myeloid and lymphoid lineage cells; NHL, non-Hodgkin lymphoma; O, osteoporosis; O + F, small-size osteolysis and/or osteoporosis with pathologic fractures; —, not detected.
With organ failure.