. Author manuscript; available in PMC: 2016 Jan 31.

Published in final edited form as: Pediatr Clin North Am. 2015 Feb;62(1):139–165. doi: 10.1016/j.pcl.2014.09.010

Table 4.

Standard treatment options for high-stage B-cell non-Hodgkin lymphoma

	Disease Stage	Disease Manifestations	Regimen	Outcome
FAB/LMB-96 International Study COG-C5961^30,113	B	Multiple extra-abdominal sites Nonresected stage I, II, III, IV Marrow <25% blasts No CNS disease	Prephase + 4 cycles of chemotherapy (reduced intensity arm)³⁰	90%–94% 4 y EFS (70% PBML)
FAB/LMB-96 International Study COG-C5961^30,113	C	Mature B-cell ALL (>25% blasts in marrow) and/or CNS disease	Prephase + 8 cycles of chemotherapy (full intensity arm)¹¹³	70%–90% EFS Best responders if in CR after 3 cycles standard intensity treatment 70% EFS in patients with CNS disease

BFM Group^10,117	R2	Nonresected stage I/II and stage III with LDH <500 IU/L	Prephase + 4 cycles of chemotherapy (4 h methotrexate infusion)¹⁰	EFS >95%
	R3	Stage III with LDH 500–999 IU/L Stage IV, B-cell ALL (>25% blasts) and LDH <1000 IU/L No CNS disease	Prephase + 5 cycles of chemotherapy (24 h methotrexate infusion)¹⁰	Overall EFS 85% for R3 Overall EFS 81% for R4 Reducing MTX from 24 to 4 h → inferior outcome (75 vs 91% EFS)
	R4	Stage III, IV, B-cell ALL with LDH >1000 IU/L Any CNS disease	Prephase + 6 cycles of chemotherapy (24 h methotrexate infusion)¹⁰	PMBL: 50% EFS (3 y) CNS presentation: 70% EFS (3 y)

Abbreviations: ALL, acute lymphoblastic leukemia; BFM, Berlin-Frankfurt-Munster; LDH, lactate dehydrogenase.