Table 2.

Clinical Characteristics of Children with A-T Who Developed Cancer

Patient No	Age (yrs)	Diagnosis	Disease sites	Stage	Treatment	Response	Outcome
1	8	DLBCL	Waldeyers ring; kidney; pelvis	III	LMB-89	CR	Alive in CCR 5 years
2	11	B-ALL	Bone marrow; CNS+	IV	LMB-89	Inevaluable	Expired secondary to relapse versus sepsis
3	9	DLBCL	Multiple nodes; CNS+	IV	LMB-89	Inevaluable	Expired secondary to enterococcal bacteremia; adenoviral sepsis; multi-organ system failure and VOD
4	6	DLBCL	Multiple nodes; liver; spleen, BM+	IV	LMB-89	Induction failure (progressive disease after COPADM #2)	Expired secondary to multi-organ system failure and induction failure
5	19	DLBCL	Waldeyers ring; mediastinum, multiple nodes	III	LMB-89	CR	Expired 3 years from diagnosis from pneumonitis

Histological subtypes included DLBCL (diffuse large B-cell lymphoma) and B-ALL (Burkitt Leukemia), Distant sites of disease included BM (bone marrow) and CNS (central nervous system). Treatment was derived from LMB-89 (French Society of Pediatric Oncology treatment regimen for mature B-cell malignancies).Responses included CR (complete remission) and CCR (continuous complete remission). Toxicity included VOD (veno-occlusive disease).