Table 1 Characteristics of patients with myeloid malignancies and the RANBP2-ALK fusion gene
Characteristic | This case | Maesako et al. 5 | Lim et al. 10 | Rottgers et al. 11 Patient 3 | Patient 4 | Patient 6 |
|---|---|---|---|---|---|---|
Diagnosis | AML (M4) | AML | AML (M4) | MDS or JMML | AML (M4) | JMML |
Karyotype | 46,XX,inv(2)(p23q13) [20] (at diagnosis) 45,XX,inv(2)(p23q13), -7 [5] (relapse) | 46, XX, inv(2)(p23q13) [1]/, 45, idem, -7 [8]/,46, idem, -7, +mar [1] | 45,XX,inv(2)(p23q13),-7 [20] | 45,XY,inv(2) (p23q13),-7 [5] | 46,XY,inv(2)(p23q13) [3]/, 45,idem,-7 [8] | 45,XY,t(2;2) (p23;q11~13),-7 |
Age at diagnosis | 2.8 Years | 75 Years | 31.4 Years | 8 Years | 16.3 Years | 3.5 Years |
Initial therapy regimen | VP-16, Ara-C, IDA | Ara-C, DNR, AZA (every 4 weeks) | Ara-C, DNR | Ara-C, DNR, VP-16 | Ara-C, DNR, VP-16 | Ara-C, DNR, VP-16 |
Response to initial therapy | Relapse 6 months after diagnosis, during consolidation therapy | Relapse after eight cycles of AZA | Induction failure | Recurrence of blasts on day 26 | Early relapse after 6 months | Low persistent blast cells ranging between 2 and 6% during the next 4 months |
Salvage therapy regimen | IDA+FLAG, AZA, Crizotinib | Crizotinib | Ara-C, MIT, VP-16 | — | liposomal DNR, FLU, Ara-C | — |
Response to crizotinib therapy | Molecular CR after 51 days crizotinib | Blasts disappeared from peripheral blood after 71 days crizotinib: blasts reappeared on day 135 | — | — | — | — |
HCT | HCT from HLA5/8 matched mother | — | MUD HCT | — | MFD HCT | MUD HCT |
Clinical outcome | Alive 1 year after HCT | No data | Relapse 3 months after HCT; death 7 months after relapse | Early death due to infection | Alive 6 years after HCT | Alive 8 years after HCT |
