Table 4 Summary of disease characteristics
From: Comprehensive mastocytosis data analysis from a single center
Diagnosis (N = 104) | n, % |
Cutaneous mastocytosis | 18 (17.31%) |
Systemic mastocytosis | 86 (82.69%) |
Indolent SM | 55 (52.88%) |
SM-AHN | 14 (13.46%) |
Smoldering SM | 6 (5.77%) |
Aggressive SM | 6 (5.77%) |
Mast cell leukemia | 5 (4.81%) |
SM-AHN type (n = 14) | n, % |
ASM-CMML | 2 (14.29%) |
ASM-MF | 2 (14.29%) |
ASM-MM | 1 (7.14%) |
ISM-CML | 1 (7.14%) |
ISM-CMML | 1 (7.14%) |
ISM-ET | 1 (7.14%) |
ISM-MGUS | 1 (7.14%) |
ISM-MZL | 1 (7.14%) |
MCL-CMML | 1 (7.14%) |
MCL-MDS | 1 (7.14%) |
SSM-MGUS | 1 (7.14%) |
SSM-PV | 1 (7.14%) |
Skin involvement, % | 33.44 ± 28.00 (0–91) |
First symptom | n (%) |
Skin lesion | 66 (63.46%) |
Anaphylaxis | 20 (19.23%) |
Constitutional symptom | 7 (6.73%) |
Abdominal pain | 2 (1.92%) |
Dyspnea | 2 (1.92%) |
Flushing | 2 (1.92%) |
Bone pain | 2 (1.92%) |
Weakness | 1 (0.96%) |
Itching | 1 (0.96%) |
Asymptomatic | 1 (0.96%) |
BM mast % (N = 86) | n (%) |
< 25% | 72 (83.72%) |
> 25% | 14 (16.28%) |
BM mast cell aggregate | 86 (82.69%) |
Scattering | 14 (16.28%) |
Patchy-scattering | 32 (37.21%) |
Nodule | 30 (34.88%) |
Diffuse | 10 (11.63%) |
BM dysplasia | n (%) |
No | 89 (85.58%) |
Rarely, dysplasia <%10 | 15 (14.42%) |
Skin symptoms | 79 (75.96%) |
Hepatomegaly | 34 (32.7%) |
Associated with hepatosteatosis | 24 (23.08%) |
Associated with mast cell disease | 10 (9.62%) |
Anaphylaxis | 30 (28.85%) |
Bone symptoms | 30 (28.85%) |
GI tract symptoms | 22 (21.15%) |
Splenomegaly | 18 (17.31%) |
Constitutional symptom | 15 (14.42%) |
CVS symptoms | 13 (12.50%) |
Lymphadenomegaly | 13 (12.50%) |
Ascites | 8 (7.69%) |
Portal hypertension | 7 (6.73%) |
X-ray (N = 92) | n (%) |
Normal | 73 (79.35%) |
Osteopenia | 4 (4.35%) |
Osteosclerosis | 8 (8.70%) |
Lytic lesion | 4 (4.35%) |
Sclerotic and lytic lesions | 3 (3.26%) |
DEXA (N = 93) | n (%) |
Normal | 18 (19.35%) |
Osteopenia (> 2.5 and < −1.0) | 50 (53.76%) |
Osteoporosis (< −2.5) | 18 (19.35%) |
Osteosclerosis | 7 (7.53%) |
Complicationsa | 18 (17.48%) |
Portal hypertension | 7 (6.80%) |
Duodenal perforation | 1 (0.97%) |
GI bleeding | 4 (3.88%) |
Compression fracture | 4 (3.88%) |
Pleural effusion | 2 (1.94%) |
Portal vein thrombosis | 1 (0.97%) |
Malignancy | 6 (5.83%) |