Table 1 Main clinico-biological features... | American Society of Hematology

Table 1

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


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
EFS	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
EFS	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.