T. Inukai et al., A NOVEL 203 KD ABERRANT BCR-ABL PRODUCT IN A GIRL WITH PHILADELPHIA-CHROMOSOME-POSITIVE ACUTE LYMPHOBLASTIC-LEUKEMIA, British Journal of Haematology, 85(4), 1993, pp. 823-825
We report a girl with Ph1-positive ALL with the aberrant BCR-ABL produ
ct. In this case, bcr exon 3 jointed not to ordinal abl exon 2 but to
exon 3 resulting in the production of a 203 kD BCR-ABL fusion protein
with marked tyrosine kinase activity. To our knowledge, this is the fi
rst report of an aberrant BCR-ABL product in childhood. This case was
characterized with younger age and low leucocyte count at the onset, b
ut relapsed early like the typical Ph1-positive ALL, suggesting the di
versity in the clinicopathogenesis of Ph1-positive ALL.