Patient characteristics: SMCD with associated eosinophilia
Cases . | 1 . | 2 . | 3 . | 4 . | 5 . |
|---|---|---|---|---|---|
| Age, y | 46 | 31 | 72 | 78 | 85 |
| Organomegaly | |||||
| Before treatment | S | H,S | S | H,S | S |
| After treatment | CR | CR | CR | No change | No change |
| Diagnosis date, BM | 05/31/01 | 06/12/01 | 2/19/03 | 06/09/98 | 10/04/02 |
| Prior treatment | None | HU, IFN-α | HU | IFN-α | HU |
| Follow-up, mos | 10+ | 19+ | 1+ | 9+ | 5+ |
| WHO classification | ISM | ASM | ASM | ASM | ASM |
| Imatinib mesylate dose: induction/maintenance, mg/d | 100, 100 | 400, 100 (biw) | 100, 100 | 100-400, NA | 100-400, NA |
| Hgb concentration, g/dL | |||||
| Before treatment | 12.1 | 9.2 | 13.9 | 10.1 | 10 |
| After treatment | 15.1 | 15.3 | 13.3 | 10.0 | 11.4 |
| WBC count, × 109/L | |||||
| Before treatment | 14.5 | 69.8 | 15.2 | 19.7 | 18 |
| After treatment | 4.9 | 6.1 | 3.1 | 21.7 | 17.5 |
| AEC count, × 109/L | |||||
| Before treatment | 10.7 | 16.1 | 10.2 | 3.5 | 6.1 |
| After treatment | 0.2 | 0.3 | 0.4 | 2.7 | 3.2 |
| Platelet count, × 109/L | |||||
| Before treatment | 118 | 31 | 213 | 210 | 166 |
| After treatment | 169 | 179 | 166 | 252 | 213 |
| Serum tryptase, less than 11.5 ng/mL | |||||
| Before treatment | 137 | ND | 56 | 904 | 331 |
| After treatment | 25.7 | ND | 19.8 | 1370 | 342 |
| c-kit Asp816Val mutation | A | A | A | P | P |
| FIP1L1-PDGFRA | P | P | P | A | A |
| CHIC2 deletion, % of nuclei positive | |||||
| Before treatment | 61.0 | 58.0 | 76.0 | 2.5 | 0.5 |
| After treatment | 0.0 | 1.5 | 0.5 | ND | ND |
| ETV6-PDGFRB | A | A | A | A | A |
| Cytogenetics | 46,XY | 46,XY | 46,XY | 46,XX | 46,XX |
| Response | Complete response | Complete response | Complete response | NR | NR |
| BM overall cellularity/mast cell % | |||||
| Before treatment | 90/30 | 95/15 | 90/20 | 90/70 | > 95/30 |
| After treatment | 35/CR | 45/CR | 60/CR | 90/70 | ND/ND |
Cases . | 1 . | 2 . | 3 . | 4 . | 5 . |
|---|---|---|---|---|---|
| Age, y | 46 | 31 | 72 | 78 | 85 |
| Organomegaly | |||||
| Before treatment | S | H,S | S | H,S | S |
| After treatment | CR | CR | CR | No change | No change |
| Diagnosis date, BM | 05/31/01 | 06/12/01 | 2/19/03 | 06/09/98 | 10/04/02 |
| Prior treatment | None | HU, IFN-α | HU | IFN-α | HU |
| Follow-up, mos | 10+ | 19+ | 1+ | 9+ | 5+ |
| WHO classification | ISM | ASM | ASM | ASM | ASM |
| Imatinib mesylate dose: induction/maintenance, mg/d | 100, 100 | 400, 100 (biw) | 100, 100 | 100-400, NA | 100-400, NA |
| Hgb concentration, g/dL | |||||
| Before treatment | 12.1 | 9.2 | 13.9 | 10.1 | 10 |
| After treatment | 15.1 | 15.3 | 13.3 | 10.0 | 11.4 |
| WBC count, × 109/L | |||||
| Before treatment | 14.5 | 69.8 | 15.2 | 19.7 | 18 |
| After treatment | 4.9 | 6.1 | 3.1 | 21.7 | 17.5 |
| AEC count, × 109/L | |||||
| Before treatment | 10.7 | 16.1 | 10.2 | 3.5 | 6.1 |
| After treatment | 0.2 | 0.3 | 0.4 | 2.7 | 3.2 |
| Platelet count, × 109/L | |||||
| Before treatment | 118 | 31 | 213 | 210 | 166 |
| After treatment | 169 | 179 | 166 | 252 | 213 |
| Serum tryptase, less than 11.5 ng/mL | |||||
| Before treatment | 137 | ND | 56 | 904 | 331 |
| After treatment | 25.7 | ND | 19.8 | 1370 | 342 |
| c-kit Asp816Val mutation | A | A | A | P | P |
| FIP1L1-PDGFRA | P | P | P | A | A |
| CHIC2 deletion, % of nuclei positive | |||||
| Before treatment | 61.0 | 58.0 | 76.0 | 2.5 | 0.5 |
| After treatment | 0.0 | 1.5 | 0.5 | ND | ND |
| ETV6-PDGFRB | A | A | A | A | A |
| Cytogenetics | 46,XY | 46,XY | 46,XY | 46,XX | 46,XX |
| Response | Complete response | Complete response | Complete response | NR | NR |
| BM overall cellularity/mast cell % | |||||
| Before treatment | 90/30 | 95/15 | 90/20 | 90/70 | > 95/30 |
| After treatment | 35/CR | 45/CR | 60/CR | 90/70 | ND/ND |
S indicates splenomegaly; CR, complete remission; H, hepatomegaly; BM, bone marrow; HU, hydroxyurea; IFN-α, interferon-alpha; WHO, World Health Organization; ISM, indolent systemic mastocytosis; ASM, aggressive systemic mastocytosis; biw, twice per week; NA, not applicable; Hgb, hemoglobin; WBC, white blood cell; AEC, absolute eosinophil; ND, not done; A, absent; P, present; and NR, no response.