Konaka Y, Namiuchi S
Department of Medicine, Kitano Hospital.
Rinsho Ketsueki. 1993 Dec;34(12):1556-61.
A 47-year-old man presented with fever, cough and chest pain in January, 1989. He was found to have mediastinal tumor and generalized lymphadenopathy. Peripheral blood and bone marrow findings were typical for the chronic phase of chronic myelogenous leukemia (CML). Although the histological findings of a cervical lymph node were indistinguishable from those of malignant lymphoma, cytogenetic studies of the lymph node cells showed positive Ph1 chromosome and rearrangement of the bcr gene as well as bone marrow cells. Double fluorescence analysis of lymph node cells demonstrated co-existence of CD5, CD7 and CD33 positive cells and of cells sharing both CD5 or CD7 and CD33 antigens. These findings suggest that tumor cells originate from the stage at which the differentiation pathways of hematopoietic stem cells branch into precursor T and myeloid cells. Various combination chemotherapies had only partial effects on lymph node swelling. Chronic daily administration of low dose etoposide was very effective to control both lymphadenopathy and leukocytosis and the patient remained well for over 2 years until July, 1991 when hematological myeloid blast crisis developed. He died of pneumonia in October, 1991. This is a rare case of CML with extramedullary mixed crisis which survived for a long time.
一名47岁男性于1989年1月出现发热、咳嗽和胸痛症状。他被发现患有纵隔肿瘤和全身淋巴结肿大。外周血和骨髓检查结果符合慢性粒细胞白血病(CML)慢性期的典型表现。尽管颈部淋巴结的组织学检查结果与恶性淋巴瘤难以区分,但淋巴结细胞的细胞遗传学研究显示存在阳性Ph1染色体以及bcr基因重排,骨髓细胞也有同样表现。淋巴结细胞的双重荧光分析表明,存在CD5、CD7和CD33阳性细胞,以及同时表达CD5或CD7和CD33抗原的细胞。这些发现提示肿瘤细胞起源于造血干细胞分化途径分支为前体T细胞和髓细胞的阶段。各种联合化疗对淋巴结肿大仅产生部分疗效。长期每日给予低剂量依托泊苷对控制淋巴结肿大和白细胞增多非常有效,该患者病情稳定超过2年,直至1991年7月发生血液学髓系原始细胞危象。他于1991年10月死于肺炎。这是一例罕见的CML合并髓外混合危象且存活较长时间的病例。