Fujie H, Tsuchiya S, Morita S, Itano M, Yambe T, Ohasi Y, Minegishi N, Minegishi M, Satou T, Konno T
Department of Pediatric Oncology, Tohoku University, Sendai, Japan.
Leukemia. 1994 Sep;8(9):1592-5.
A 13-year-old boy without any previous illness was diagnosed as suffering from acute lymphoblastic leukemia (ALL). After a period of apparent complete remission until 17 years of age, the presence of Ph1 positive cells in bone marrow was demonstrated by karyotype analysis. This finding suggested chronic myelogenous leukemia (CML) because of the absence of blastic changes in bone marrow but mild leukocytosis with basophilia at that time. Six months later he had a relapse (blast crisis) with the appearance of peroxidase negative lymphoid blasts and myeloid surface markers. To make differential diagnosis, leukemia blasts at onset and relapse were examined for rearrangement of immunoglobulin JH gene and bcr/abl fusion mRNA, and were found to have the same JH gene rearrangement pattern and the same bcr/abl mRNA of bcr exon 2/abl exon 2. These results indicate an unusual case of CML which appeared in blast crisis at onset, followed by a long-term remission.
一名既往无任何疾病的13岁男孩被诊断为急性淋巴细胞白血病(ALL)。在经历一段明显的完全缓解期直至17岁后,通过核型分析证实骨髓中存在Ph1阳性细胞。这一发现提示为慢性粒细胞白血病(CML),原因是当时骨髓中无原始细胞变化,但有轻度白细胞增多伴嗜碱性粒细胞增多。6个月后,他出现复发(原始细胞危象),伴有过氧化物酶阴性的淋巴母细胞和髓系表面标志物。为进行鉴别诊断,对发病时和复发时的白血病原始细胞进行免疫球蛋白JH基因重排和bcr/abl融合mRNA检测,发现具有相同的JH基因重排模式以及相同的bcr外显子2/abl外显子2的bcr/abl mRNA。这些结果表明这是一例不寻常的CML病例,发病时即出现原始细胞危象,随后是长期缓解。