Heim S, Christensen B E, Fioretos T, Sørensen A G, Pedersen N T
Department of Medical Genetics, Odense University Hospital, Denmark.
Cancer Genet Cytogenet. 1992 Mar;59(1):35-8. doi: 10.1016/0165-4608(92)90154-z.
The reciprocal translocation (9;22)(q34;q11) is highly characteristic of chronic myeloid leukemia (CML) and the pericentric inversion inv(16)(p13q22) is almost only found in acute nonlymphocytic leukemia of the myelomonocytic subtype (ANLL M4). Only twice before have an inv(16) and a t(9;22) been found in the same cells, and both times the patients seemed to have de novo ANLL M4. We describe the case of a 21-year-old man who in July 1986 presented with a clinically and hematologically classic chronic phase CML. Treatment with busulfan led to no improvement; instead in September 1986 he developed blast crisis with ANLL M4Eo morphology. He was now cytogenetically examined and the karyotype 45,X,-Y,t(9;22)(q34;q11),inv(16)(p13q22) was found. Southern blot analysis of the bone marrow DNA sampled at this time revealed a standard rearrangement in the 3' end of the M-bcr. Intensive cytostatic treatment caused cytopenia followed by complete hematologic, clinical, and cytogenetic reversal to chronic phase CML, so that in January 1987 the bone marrow karyotype was 46,XY,t(9;22)(q34;q11). Persistent splenomegaly was treated with splenectomy, and a chloroma of the skin was removed by irradiation. In March 1987 he received an allogeneic bone marrow transplant. Since then his only medical problem has been mild graft-versus-host disease; he is well and is working full time as a blacksmith.
相互易位(9;22)(q34;q11)是慢性粒细胞白血病(CML)的高度特征性表现,而臂间倒位inv(16)(p13q22)几乎仅见于髓单核细胞亚型的急性非淋巴细胞白血病(ANLL M4)。此前仅两次在同一细胞中发现inv(16)和t(9;22),且两次患者似乎均为初发ANLL M4。我们报告一例21岁男性病例,该患者于1986年7月出现临床和血液学表现典型的慢性期CML。白消安治疗无效;相反,1986年9月他发展为具有ANLL M4Eo形态的急变期。此时对他进行细胞遗传学检查,发现核型为45,X,-Y,t(9;22)(q34;q11),inv(16)(p13q22)。对此时采集的骨髓DNA进行Southern印迹分析,发现在M-bcr的3'端有标准重排。强化的细胞抑制治疗导致血细胞减少,随后血液学、临床和细胞遗传学完全逆转至慢性期CML,因此1987年1月骨髓核型为46,XY,t(9;22)(q34;q11)。持续性脾肿大通过脾切除术治疗,皮肤绿色瘤通过放疗切除。1987年3月他接受了异基因骨髓移植。从那以后,他唯一的医疗问题就是轻度移植物抗宿主病;他情况良好,作为一名铁匠全职工作。