Saigo K, Sugimoto T, Ryo R, Yamaguchi N
Section of Internal Medicine, Kobe Kyodo Hospital.
Rinsho Ketsueki. 1995 Jan;36(1):18-22.
An 82-year-old female was diagnosed as having 5q- syndrome accompanied by basophilia, eosinophilia and thrombocytosis. Since cytogenetic abnormalities other than 5q- were also detected, she was considered to be type B of 5q- syndrome. According to the FAB classification a diagnosis of refractory anemia with excess of blasts (RAEB) was made. She was treated with recombinant interferon alpha 2b, because peripheral blood findings resembled those of chronic myelogenous leukemia. Interferon was effective, and resulted in disappearance of peripheral blasts, normalization of platelet numbers, and improvement of basophilia. These changes were interferon-dependent. After 1 year, cytogenetic studies revealed that about 2/3 of metaphases showed normal karyotype. Twenty months after diagnosis, myeloid blastic crisis occurred and eventually the patient died. However, treatment with interferon in this case might support the usefulness of the drugs for this kind of disease.
一名82岁女性被诊断为伴有嗜碱性粒细胞增多、嗜酸性粒细胞增多和血小板增多的5q-综合征。由于还检测到了除5q-之外的细胞遗传学异常,她被认为是5q-综合征的B型。根据FAB分类,诊断为难治性贫血伴原始细胞增多(RAEB)。由于外周血表现类似于慢性粒细胞白血病,她接受了重组干扰素α 2b治疗。干扰素治疗有效,导致外周原始细胞消失、血小板数量恢复正常以及嗜碱性粒细胞增多情况改善。这些变化依赖于干扰素。1年后,细胞遗传学研究显示约2/3的中期细胞显示核型正常。诊断20个月后,发生了髓系原始细胞危象,患者最终死亡。然而,该病例中使用干扰素治疗可能支持这类药物对这种疾病的有效性。