Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osaka-Sayama, Japan.
Front Immunol. 2019 Jan 9;9:3031. doi: 10.3389/fimmu.2018.03031. eCollection 2018.
Myelodysplastic syndromes (MDS) are a heterogeneous group clonal disorders of hematopoietic stem cells (HSC) characterized by ineffective hematopoiesis that lead to variable grades of impaired blood cell production. Chromosomal aberrations are often detected in MDS patients and thus cytogenetic analysis is useful for the diagnosis of these disorders. Common recurring chromosomal defects, such as the -5/5q- and -7/7q- are relatively well characterized cytogenetic abnormalities in MDS, however, the biological significance of uncommon cytogenetic alterations is unknown. We report here, two cases of peripheral blood and bone marrow hypereosinophilia in patients with MDS harboring the unbalanced translocation der(1;7)(q10;p10), a poorly characterized cytogenetic abnormality that is found in certain myeloid malignancies, including MDS. The patients reported here presented hypereosinophilia that was refractory to steroids and cytotoxic therapy, leading to severe target tissue damage that ultimately resulted in fatal end-organ failure. Potential roles of the der(1;7)(q10;p10) aberrations in the pathogenesis of aggressive eosinophilia and disease prognosis are discussed here.
骨髓增生异常综合征(MDS)是一组异质性克隆性造血干细胞(HSC)疾病,其特征为无效造血,导致血细胞生成的不同程度受损。MDS 患者常检测到染色体异常,因此细胞遗传学分析对这些疾病的诊断很有用。常见的重现性染色体缺陷,如-5/5q-和-7/7q-,是 MDS 中相对特征明确的细胞遗传学异常,但不常见的细胞遗传学改变的生物学意义尚不清楚。我们在这里报告两例 MDS 患者外周血和骨髓嗜酸性粒细胞增多,这些患者携带有不平衡易位 der(1;7)(q10;p10),这是一种特征不明确的细胞遗传学异常,存在于某些髓系恶性肿瘤中,包括 MDS。这里报告的患者表现出对类固醇和细胞毒性治疗有抗性的嗜酸性粒细胞增多症,导致严重的靶组织损伤,最终导致致命的终末器官衰竭。这里讨论了 der(1;7)(q10;p10)异常在侵袭性嗜酸性粒细胞增多症和疾病预后中的潜在作用。