Table 1

Main clinico-biological features of t(4;11) MA4+ B-ALL

CharacteristicComment
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 
CharacteristicComment
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 

EFS, event-free survival; FLT3, fms-like tyrosine kinase 3; RAS, rat sarcoma; WBC, white blood cell.

*

Estimated in Europe/United States.

Based on Interfant protocol.

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