费城阳性非典型慢性髓性白血病,BCR/ABL阴性1例报告

Germán Rivera Monroy, Federica Riccio Cuomo, Dunia Michelle Bustos Campa, Jorge Luis López Marthen
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摘要

非典型髓系白血病是一种骨髓增生性肿瘤,患者骨髓细胞增多导致髓系血细胞增生异常。我们的病人呈现阳性的Ph核型和阴性的ABL/BCR转录。此外,她的年龄不符合诊断的平均年龄。她的第一次治疗是羟基脲1 g/kg,持续60天,对疾病的发展没有积极作用,因此选择治疗改为伊马替尼400 mg/天。两个月后,治疗产生了良好的反应,这使得我们认为伊马替尼治疗比其他常规治疗有更好的临床效果。本文介绍了在曼纽尔Ávila卡马乔国家医疗中心进行的研究,并将其与现有的关于诊断和治疗的文献进行比较,以评估相似性并突出可能有助于未来病例管理的具体要点。
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Philadelphia-positive atypical chronic myeloid leukemia, BCR/ABL negative: a case report
Atypical myeloid leukemia is a myeloproliferative neoplasm in which patients have a dysplastic increase in blood cells of the myeloid lineage as a result of hypercellular bone marrow. Our patient presented a positive Ph karyotype and a negative ABL/BCR transcript. Moreover, her age does not correspond to the mean of diagnosis. Her first treatment was hydroxyurea 1 g/kg for 60 days, which was not positive for the evolution of the disease and thus the elective treatment was changed to imatinib 400 mg/ day. After two months, the treatment has produced a favorable response, which leads to think that the therapy with imatinib offers better clinical results than other conventional ones. This text presents the study carried out at the Manuel Ávila Camacho National Medical Center and compares it with the existing literature regarding diagnosis and treatment to assess similarities and highlight specific points that may help in future case management.
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