Summary of representative studies (series consisting of 4 patients or more) that describe use of imatinib in the treatment of primary eosinophilic disorders
Study . | Clinical diagnosis . | No. patients . | Cytogenetic findings . | c-kit or PDGFR mutations . | Clinical response . | Molecular or cytogenetic response . | Imatinib dose, mg/d . |
|---|---|---|---|---|---|---|---|
| Gleich et al70 | HES | 5 | 3 normal | Negative for c-kit D816V (n = 4) | 4 CR | NA | 100 |
| 2 ND | 1 NR | ||||||
| Pardanani et al73 | HES/CMPD-eos | 7 | 6 normal | Negative for c-kit and PDGFRB mutations (n = 6) | 3 CR | Patient with +8 did not respond | 100-400 |
| 1 trisomy 8 | 1 PR | 100-400 | |||||
| 3 NR | |||||||
| Cortes et al72 | HES | 9 | All normal | ND | 4 CR | NA | 100-400 |
| 1 PR | |||||||
| 4 NR | |||||||
| Apperley et al16 | CMPD-eos | 4 | t(5;12)(q33;p13) in all 4 patients | ETV6/PDGFRB (n = 3) | 4 CR | 4 CR | 400 |
| Cools et al11 | HES, AML | 11 | 9 normal | FIP1L1/PDGFRA in 5 of 10 tested, including the 2 patients with abnormal cytogenetics | 9 CR | ND | 100-400 |
| 1 t(1;4)(q44;q12) | 2 NR | ||||||
| 1 trisomy 8 and 19 | |||||||
| Klion et al45 | MHES | 6 | All normal | FIP1L1/PDGFRA in all 6 patients | 6 CR | ND | 400 |
| Pardanani et al14 | SM-eos | 5 | All normal | FIP1L1/PDGFRA (n = 3) c-kit D816V (n = 2) | 3 CR | 3 CR | 100-400 |
| 2 NR | |||||||
| Klion et al17 | MHES | 7 | All normal | FIP1L1/PDGFRA in all 7 patients | 7 CR | 5 CR (n = 6) | 300-400 |
| Vandenberghe et al46 | CEL | 4 | All normal | FIP1L1/PDGFRA in all 4 patients | 4 CR | 2 CR | 100 |
| Salem et al79 | HES | 6 | 5 normal | Negative for ETV6/PDGFRB | 6 CR | NA | 100 |
| 1 trisomy 8 |
Study . | Clinical diagnosis . | No. patients . | Cytogenetic findings . | c-kit or PDGFR mutations . | Clinical response . | Molecular or cytogenetic response . | Imatinib dose, mg/d . |
|---|---|---|---|---|---|---|---|
| Gleich et al70 | HES | 5 | 3 normal | Negative for c-kit D816V (n = 4) | 4 CR | NA | 100 |
| 2 ND | 1 NR | ||||||
| Pardanani et al73 | HES/CMPD-eos | 7 | 6 normal | Negative for c-kit and PDGFRB mutations (n = 6) | 3 CR | Patient with +8 did not respond | 100-400 |
| 1 trisomy 8 | 1 PR | 100-400 | |||||
| 3 NR | |||||||
| Cortes et al72 | HES | 9 | All normal | ND | 4 CR | NA | 100-400 |
| 1 PR | |||||||
| 4 NR | |||||||
| Apperley et al16 | CMPD-eos | 4 | t(5;12)(q33;p13) in all 4 patients | ETV6/PDGFRB (n = 3) | 4 CR | 4 CR | 400 |
| Cools et al11 | HES, AML | 11 | 9 normal | FIP1L1/PDGFRA in 5 of 10 tested, including the 2 patients with abnormal cytogenetics | 9 CR | ND | 100-400 |
| 1 t(1;4)(q44;q12) | 2 NR | ||||||
| 1 trisomy 8 and 19 | |||||||
| Klion et al45 | MHES | 6 | All normal | FIP1L1/PDGFRA in all 6 patients | 6 CR | ND | 400 |
| Pardanani et al14 | SM-eos | 5 | All normal | FIP1L1/PDGFRA (n = 3) c-kit D816V (n = 2) | 3 CR | 3 CR | 100-400 |
| 2 NR | |||||||
| Klion et al17 | MHES | 7 | All normal | FIP1L1/PDGFRA in all 7 patients | 7 CR | 5 CR (n = 6) | 300-400 |
| Vandenberghe et al46 | CEL | 4 | All normal | FIP1L1/PDGFRA in all 4 patients | 4 CR | 2 CR | 100 |
| Salem et al79 | HES | 6 | 5 normal | Negative for ETV6/PDGFRB | 6 CR | NA | 100 |
| 1 trisomy 8 |
CR indicates complete response; NR, no response; PR, partial response; MHES, myeloproliferative variant of HES; eos-CMPD, eosinophilia associated chronic myeloproliferative disorder; D816V, aspartate 816 to valine mutation; ND, not done or not reported; NA, not applicable. Remaining abbreviations are explained in Table 1.