Table 3. B and C findings in systemic mastocytosis (SM).
BM: Bone marrow, ANC: Absolute neutrophil count
B findings | C findings |
1. High mast cell burden shown on BM biopsy: >30% infiltration of cellularity by mast cells (focal, dense aggregates) and serum total tryptase level > 200 ng/mL | 1. Bone marrow dysfunction caused by neoplastic mast cell infiltration, manifested by ≥1 cytopenia(s) (ANC <1.0 × 109/L, Hgb <10 g/dL, and/or platelet count <100 × 109/L). |
2. High mast cell burden shown on BM biopsy: >30% infiltration of cellularity by mast cells (focal, dense aggregates) and serum total tryptase level > 200 ng/mL | 2. Palpable hepatomegaly with impairment of liver function, ascites and/or portal hypertension. |
3. Signs of dysplasia or myeloproliferation, in non-mast cell lineage(s), but insufficient criteria for definitive diagnosis of an associated hematological neoplasm (AHN), with normal or only slightly abnormal blood counts. | 3. Skeletal involvement with large osteolytic lesions with/without pathological fractures (pathological fractures caused by osteoporosis do not qualify as a “C” finding. |
4. Hepatomegaly without impairment of liver function, palpable splenomegaly without hypersplenism, and/or lymphadenopathy on palpation or imaging. | 4. Palpable splenomegaly with hypersplenism. |
5. Malabsorption with weight loss due to gastrointestinal mast cell infiltrates. |