Main clinico-biological features of t(4;11) MA4+ B-ALL
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Characteristic . | Comment . |
Incidence* | Estimated 1 case/1 million newborns |
Phenotype | Pro-B/mixed: CD34+CD19+CD10− with expression of the myeloid markers CD15 and CD65 and NG2. |
EFS | 5-year EFS is 34% for Children’s Cancer Group protocol CCG-1953 |
4-year EFS is 37% for Interfant-99 protocol | |
State of the art treatment† | Initial glucocorticoid-based treatment (prednisone, l-asparaginase), followed by induction (dexamethasone, cytarabine, vincristine, daunorubicin, and mitoxantrone) and by myeloid-like (cytarabine, daunorubicin/mitoxantrone, and etoposide) or lymphoid-like consolidation (cyclophosphamide, cytarabine, and 6-mercaptopurine) |
CNS involvement† | Common: up to 50% accumulative at diagnosis and during evolution |
Age at diagnosis | <1 year |
Negative prognostic factors | High WBC counts (>300 000 WBC/µL) |
Age < 6 months | |
CNS infiltration | |
Early poor response to prednisone | |
RAS mutations | |
High expression level of FLT3 | |
Low expression level of HoxA cluster |
. | . |
---|---|
Characteristic . | Comment . |
Incidence* | Estimated 1 case/1 million newborns |
Phenotype | Pro-B/mixed: CD34+CD19+CD10− with expression of the myeloid markers CD15 and CD65 and NG2. |
EFS | 5-year EFS is 34% for Children’s Cancer Group protocol CCG-1953 |
4-year EFS is 37% for Interfant-99 protocol | |
State of the art treatment† | Initial glucocorticoid-based treatment (prednisone, l-asparaginase), followed by induction (dexamethasone, cytarabine, vincristine, daunorubicin, and mitoxantrone) and by myeloid-like (cytarabine, daunorubicin/mitoxantrone, and etoposide) or lymphoid-like consolidation (cyclophosphamide, cytarabine, and 6-mercaptopurine) |
CNS involvement† | Common: up to 50% accumulative at diagnosis and during evolution |
Age at diagnosis | <1 year |
Negative prognostic factors | High WBC counts (>300 000 WBC/µL) |
Age < 6 months | |
CNS infiltration | |
Early poor response to prednisone | |
RAS mutations | |
High expression level of FLT3 | |
Low expression level of HoxA cluster |