Mann Georg, Trebo Monika M, Haas Oskar A, Grümayer-Panzer Eva R, Dworzak Michael N, Lion Thomas, Gadner Helmut
Children's Cancer Research Institute, St. Anna Children's Hospital, Kinderspitalgasse 6, 1090 Vienna, Austria.
Br J Haematol. 2002 Aug;118(2):559-62. doi: 10.1046/j.1365-2141.2001.03598.x.
Philadelphia chromosome-positive (Ph+) acute leukaemia usually shows lymphoblastic morphology and a B-precursor phenotype. The bone marrow aspirate of a 9-year-old boy showed a L3 blast cell morphology in 90% of cells; immunophenotyping revealed a mature B-blast population. The translocation t(9;22) (q34;q11) was seen in 45 out of 50 metaphases, and expression of the corresponding bcr1/abl fusion transcripts, but no IgH/myc co-localization or splitting of c-myc, was demonstrated. Chemotherapy according to the Berlin-Frankfurt-Munster non-Hodgkin's lymphoma (NHL-BFM 95) protocol with maintenance according to the BFM acute lymphoblastic leukaemia (ALL-BFM 90) protocol resulted in continuing complete remission of 54 months. The occurrence of Ph+ Burkitt's leukaemia might reflect multiple-step cancer development.
费城染色体阳性(Ph+)急性白血病通常表现为淋巴细胞形态和B前体表型。一名9岁男孩的骨髓穿刺显示90%的细胞为L3原始细胞形态;免疫表型分析显示为成熟B原始细胞群体。在50个中期细胞中有45个可见t(9;22)(q34;q11)易位,并且证实了相应bcr1/abl融合转录本的表达,但未发现IgH/myc共定位或c-myc分裂。按照柏林-法兰克福-明斯特非霍奇金淋巴瘤(NHL-BFM 95)方案进行化疗,并根据BFM急性淋巴细胞白血病(ALL-BFM 90)方案进行维持治疗,使完全缓解持续了54个月。Ph+伯基特白血病的发生可能反映了癌症的多步骤发展。