Balatzenko Gueorgui, Stoyanov Nikolay, Bekrieva Elena, Guenova Margarita
Laboratory of Cytogenetics & Molecular Biology;
Hematol Rep. 2011 Aug 31;3(2):e17. doi: 10.4081/hr.2011.e17. Epub 2011 Oct 11.
We present for the first time a 40-year-old male patient with a 20 year history of occupational exposure to radiation as a nuclear power plant worker, who developed FIP1L1-PDGFRA-positive chronic eosinophilic leukemia 27 months after radiotherapy for testicular seminoma. After an one-year history of dry cough, itching and night sweats, the patient presented with an elevated leukocyte count with absolute eosinophilia of 14.2×10(9)/L, bone marrow and lymph node involvement. Treatment with Imatinib was initiated, resulting in complete hematological remission at the sixth month and complete molecular response by nested primers reverse transcription polymerase chain reaction - at the end of the first year. This case contributes to the clinical heterogeneity of a rare entity such as FIP1L1-PDGFA-positive myeloproliferative neoplasms, and for the possible role of occupational and therapeutic radiation, raising the question if one or both of them might be the causative factor.
我们首次报告了一名40岁男性患者，他作为核电站工人有20年职业性辐射暴露史，在睾丸精原细胞瘤放疗27个月后发生了FIP1L1-PDGFRA阳性慢性嗜酸性粒细胞白血病。在有干咳、瘙痒和盗汗1年病史后，患者出现白细胞计数升高，绝对嗜酸性粒细胞增多至14.2×10⁹/L，有骨髓和淋巴结受累。开始使用伊马替尼治疗，在第6个月时实现完全血液学缓解，在第1年末通过巢式引物逆转录聚合酶链反应实现完全分子反应。该病例有助于罕见实体如FIP1L1-PDGFA阳性骨髓增殖性肿瘤的临床异质性研究，以及职业性和治疗性辐射可能发挥的作用，引发了其中一个或两者是否可能是致病因素的问题。
