Matsuda Mitsuhiro, Morita Yasuyoshi, Shimada Takahiro, Miyatake Junichi, Hirase Chikara, Tanaka Miyako, Tatsumi Yoichi, Maeda Yasuhiro, Kanamaru Akihisa
Division of Hematology, Nephrology and Rheumatology, Department of Internal Medicine, Kinki University School of Medicine, Osaka 589-8511, Japan.
Int J Hematol. 2005 May;81(4):307-9. doi: 10.1532/IJH97.04188.
We describe a patient with Philadelphia chromosome (Ph)-positive chronic myelogenous leukemia (CML) who developed an extramedullary blast crisis in the central nervous system (CNS) and then a subcutaneous tumor of the neck during treatment with imatinib mesylate. Administered 400 mg of imatinib mesylate after the diagnosis of chronic-phase CML, the patient achieved a complete cytogenetic remission 4 months later. However, he developed a mixed myeloid/B-cell blast crisis with additional karyotype abnormalities only in the CNS during a complete cytogenetic remission in the bone marrow. Several doses of intrathecal chemotherapy and whole-brain irradiation were effective in treating the blast crisis in the CNS. After 7 months of complete cytogenetic remission, the patient experienced a subcutaneous tumor in the right neck. A biopsy of the tumor revealed a mixed myeloid/T-cell blast crisis. The cytogenetic analysis showed that the blast crisis clone in the neck tumor was different from that of the CNS. An increased dose of imatinib mesylate was ineffective in treating the neck tumor. Irradiation to the right neck was therefore undertaken. This case suggests that the development of a clone resistant to imatinib mesylate is not always detected in the bone marrow and that multiple Ph-positive clones have the potential to become transformed into a blast crisis.
我们描述了一名费城染色体（Ph）阳性慢性髓性白血病（CML）患者，该患者在接受甲磺酸伊马替尼治疗期间，中枢神经系统（CNS）发生髓外原始细胞危象，随后颈部出现皮下肿瘤。慢性期CML诊断后给予400mg甲磺酸伊马替尼，患者4个月后实现完全细胞遗传学缓解。然而，在骨髓完全细胞遗传学缓解期间，他仅在CNS发生了伴有额外核型异常的混合髓系/ B细胞原始细胞危象。几剂鞘内化疗和全脑照射有效治疗了CNS的原始细胞危象。在完全细胞遗传学缓解7个月后，患者右颈部出现皮下肿瘤。肿瘤活检显示为混合髓系/ T细胞原始细胞危象。细胞遗传学分析表明，颈部肿瘤中的原始细胞危象克隆与CNS中的不同。增加剂量的甲磺酸伊马替尼对治疗颈部肿瘤无效。因此对右颈部进行了照射。该病例表明，对甲磺酸伊马替尼耐药的克隆并非总是在骨髓中被检测到，并且多个Ph阳性克隆有可能转化为原始细胞危象。
