Laboratory of Cytogenetics, Pasteur Institute of Morocco, Casablanca, Morocco.
Laboratory of Physiopathology and Molecular Genetics, Faculty of Sciences Ben M'Sik, Hassan II University, Casablanca, Morocco.
Ann Hematol. 2024 Aug;103(8):2765-2774. doi: 10.1007/s00277-024-05747-3. Epub 2024 Apr 24.
Chronic myeloid leukemia (CML) is a neoplastic disease of genetic origin resulting from clonal proliferation of hematopoietic stem cells (HSCs). The reciprocal translocation t(9;22)(q34;q11) is the main chromosomal abnormality involved in this pathology, usually detected by conventional cytogenetics. This article aims to investigate the epidemiological, cytogenetic, therapeutic, and clinical characteristics of Moroccan patients with CML. This research represents the first large-scale study of CML patients in Morocco and was carried out at Institut Pasteur of Morocco. Bone marrow samples were processed for cytogenetic analysis, and karyotypes were described according to an international system of human cytogenetic nomenclature (ISCN 2016). Patients were studied according to their epidemiological characteristics, clinical information and cytogenetic results. For statistical calculations, R version 4.3.1 was used to analyze the data and calculate the statistical parameters. RStudio and Power BI were used for data visualization. The National Cancer Institute (NCI) Surveillance, Epidemiology, and End Results (SEER) method of incidence estimation was used to calculate our incidence. We received 826 patients (from 1992 to 2023) who were referred for suspected CML or who were undergoing treatment. Only 650 patients with confirmed CML were included in the study, all of whom underwent their first cytogenetic test. The median age of our patients was 45 years and the sex ratio was 1.03. At the time of diagnosis, 147 (30%) of the patients had clinical manifestations. Most patients were diagnosed in the chronic phase (94.5%). Nineteen complex variant translocations of the Philadelphia (Ph) chromosome were detected. At the time of diagnosis, 55 (11.5%) patients had ACAs, of which 30 (54.5%) were high-risk ACAs. Based on data from 174 patients treated with imatinib, the median time to complete cytogenetic response (CCyR) was 11 months, and at the last cytogenetic follow-up, 81 patients (46.6%) achieved CCyR, while 64 patients (36.8%) showed no response to treatment. Regarding adherence to European LeukemiaNet (ELN) guidelines, 58 patients (33%) were followed according to these guidelines, with optimal treatment in 8.6%, suboptimal treatment in 7% and treatment failure in 18%. The estimated incidence of chronic myeloid leukemia calculated is 0.6 cases per 100,000 in the Casablanca region. This study provides a detailed overview of CML in Morocco, highlighting important clinical, cytogenetic and therapeutic aspects despite some limitations. It also highlights the need to deepen our understanding of this complex disease for disease management in our specific context.
慢性髓细胞白血病(CML)是一种遗传性肿瘤疾病,源于造血干细胞(HSCs)的克隆性增殖。9 号和 22 号染色体(q34;q11)的相互易位 t(9;22)是涉及这种病理学的主要染色体异常,通常通过常规细胞遗传学检测到。本文旨在研究摩洛哥 CML 患者的流行病学、细胞遗传学、治疗和临床特征。这项研究代表了摩洛哥首例大规模 CML 患者研究,是在摩洛哥巴斯德研究所进行的。对骨髓样本进行细胞遗传学分析,并根据国际人类细胞遗传学命名体系(ISCN 2016)描述核型。根据患者的流行病学特征、临床信息和细胞遗传学结果进行研究。为了进行统计计算,使用 R 版本 4.3.1 分析数据并计算统计参数。使用 RStudio 和 Power BI 进行数据可视化。使用美国国家癌症研究所(NCI)的监测、流行病学和结果(SEER)方法估计发病率来计算我们的发病率。我们收到了 826 名(1992 年至 2023 年)疑似 CML 患者或正在接受治疗的患者。仅对 650 名经确认患有 CML 的患者进行了研究,所有患者均接受了首次细胞遗传学检测。我们患者的中位年龄为 45 岁,性别比为 1.03。在诊断时,147 名(30%)患者有临床表现。大多数患者被诊断为慢性期(94.5%)。检测到 19 种复杂的费城(Ph)染色体变体易位。在诊断时,55 名(11.5%)患者有急性细胞减少症,其中 30 名(54.5%)为高危急性细胞减少症。根据 174 名接受伊马替尼治疗的患者的数据,完全细胞遗传学缓解(CCyR)的中位时间为 11 个月,在最后一次细胞遗传学随访时,81 名(46.6%)患者达到 CCyR,而 64 名(36.8%)患者对治疗无反应。关于遵守欧洲白血病网络(ELN)指南,58 名(33%)患者根据这些指南进行了随访,其中 8.6%接受了最佳治疗,7%接受了次优治疗,18%治疗失败。计算得出的慢性髓细胞白血病的发病率为每 10 万人中有 0.6 例在卡萨布兰卡地区。本研究详细介绍了摩洛哥的 CML,突出了重要的临床、细胞遗传学和治疗方面,尽管存在一些局限性。它还强调了需要加深对这种复杂疾病的了解,以便在我们的特定环境中进行疾病管理。