Demographic and clinical characteristics at referral of 342 patients with SM
| Characteristic . | No. of patients (%) . | Median (range) . |
|---|---|---|
| Total no. of SM patients | 342 | |
| ISM | 159 (46) | |
| Isolated bone marrow mastocytosis | 36 (23) | |
| Smoldering systemic mastocytosis | 22 (14) | |
| ASM | 41 (12) | |
| SM-AHNMD | 138 (40) | |
| MCL | 4 (1) | |
| Male | 188 (55) | |
| Age, y | 57 (19-87) | |
| Time from appearance of symptoms to diagnosis of SM, mo | 33 (0-516) | |
| Follow-up, mo | ||
| From onset of symptoms | 69 (1.6-741.1) | |
| From diagnosis of SM | 20.7 (0-417.1) | |
| Clinical findings | ||
| Urticaria pigmentosa | 140 (41) | |
| Cutaneous symptoms* | 182 (53) | |
| Constitutional symptoms† | 142 (42) | |
| Mediator-related symptoms‡ | 160 (47) | |
| Idiopathic and/or recurrent anaphylactoid reaction | 57 (17) | |
| Musculoskeletal symptoms§ | 107 (31) | |
| Gastrointestinal symptoms‖ | 221 (65) | |
| Hepatosplenomegaly | ||
| Hepatomegaly, n = 342 | 92 (27) | |
| Splenomegaly, n = 335 | 123 (37) | |
| Both hepatomegaly and splenomegaly, n = 335 | 72 (21) | |
| Lymphadenopathy¶ | 73 (21) | |
| Leukemic transformation (AML or MCL) | 21 (6) |
| Characteristic . | No. of patients (%) . | Median (range) . |
|---|---|---|
| Total no. of SM patients | 342 | |
| ISM | 159 (46) | |
| Isolated bone marrow mastocytosis | 36 (23) | |
| Smoldering systemic mastocytosis | 22 (14) | |
| ASM | 41 (12) | |
| SM-AHNMD | 138 (40) | |
| MCL | 4 (1) | |
| Male | 188 (55) | |
| Age, y | 57 (19-87) | |
| Time from appearance of symptoms to diagnosis of SM, mo | 33 (0-516) | |
| Follow-up, mo | ||
| From onset of symptoms | 69 (1.6-741.1) | |
| From diagnosis of SM | 20.7 (0-417.1) | |
| Clinical findings | ||
| Urticaria pigmentosa | 140 (41) | |
| Cutaneous symptoms* | 182 (53) | |
| Constitutional symptoms† | 142 (42) | |
| Mediator-related symptoms‡ | 160 (47) | |
| Idiopathic and/or recurrent anaphylactoid reaction | 57 (17) | |
| Musculoskeletal symptoms§ | 107 (31) | |
| Gastrointestinal symptoms‖ | 221 (65) | |
| Hepatosplenomegaly | ||
| Hepatomegaly, n = 342 | 92 (27) | |
| Splenomegaly, n = 335 | 123 (37) | |
| Both hepatomegaly and splenomegaly, n = 335 | 72 (21) | |
| Lymphadenopathy¶ | 73 (21) | |
| Leukemic transformation (AML or MCL) | 21 (6) |
ISM indicates indolent systemic mastocytosis (SM); ASM, aggressive SM; SM-AHNMD, SM with associated clonal hematologic non–mast cell lineage disease; MCL, mast cell leukemia; AML, acute myeloid leukemia; and No., number.
Includes pruritus, flushing, urticaria, and angioedema.
Includes weight loss, fever, chills, and night sweats.
Includes headache, dizziness/lightheadedness, syncope/presyncope, hypotension, anaphylaxis, palpitation/tachycardia, bronchoconstriction/wheezing, and peptic ulcer disease.
Includes bone pain, arthralgias, and myalgias.
Includes nausea/vomiting, dyspepsia, dysphagia, diarrhea, constipation, abdominal pain/cramping, bloating/flatulence, early satiety, heartburn, gastrointestinal tract bleeding, malabsorption, and steatorrhea.
Either palpable or detected by imaging studies.