A novel 203 kD aberrant BCR-ABL product in a girl with Philadelphia chromosome positive acute lymphoblastic leultaemia |
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Authors: | Takeshi Lnukai Kanji Sugita Toshio Suzuki Kiyomu Ijima Kumiko Goi Toru Tezuka Satoru Kojika Kazuo Hatakeyama Keiko Kagami Taijiro Mori Toshiko Okazaki Shuki Mizutani Shinpei Nakazawa |
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Affiliation: | Departrment of Paediatrics. Yamanashi Medical College, Yamanashi;Department cf Paediatrics, School of Medicine. Keio University, Tokyo;Laboratory of Immunology, Saitatma Central Hospital, Saitama;Department of Virology. The National Children's Medical Research Centre, Tokyo. Japan |
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Abstract: | Summary We report a girl with Ph1-positive ALL with the aberrant BCR-ABL product. In this case. bcr exon 3 jointed not to ordinal abl exon 2 but to exon 3 resulting in the production of a 20 3 kD BCR-ABL fusion protein with marked tyrosine kinase activity. To our knowledge, this is the first report of an aberrant BCR-ABL product in childhood. This case was characterized with younger age and low leucocyte count at the onset, but relapsed early like the typical Phl-positive ALL, suggesting the diversity in the clinicopathogenesis of Ph1-positive ALL. |
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