Gologan R, Ostroveanu Daniela, Dobrea Camelia, Gioadă Liliana
Clinic of Hematology, Fundeni Clinical Institute, Bucharest, Romania.
Rom J Intern Med. 2003;41(4):447-55.
The apparent contradiction between clonal expansion and marrow failure encountered in myelodysplastic syndromes (MDS) is more evident in hypocellular forms at presentation. Hypoplastic MDS (hMDS) appears to be a distinct clinicopathologic entity, accounting for about 15% from all MDS. The pathogeny is supposed to result from immunosupressive mechanisms and some observations on successful treatment with Cyclosporine A (CsA) are reported. The case of a young female patient diagnosed by bone marrow core biopsy with hMDS - refractory anemia (FAB and WHO classification) with normal karyotype and scarce CD34(+) cells by immunohistophenotyping is presented. She was treated with androgens followed by CsA for a few months and shortly after she developed an acute myeloid leukemia (M4) which responded to low-doses of daily oral melphalan. This is one of the first few reports on such an event during the immunosuppressive therapy in MDS and the possible explanations for this unusual evolution are discussed.
骨髓增生异常综合征（MDS）中克隆性增殖与骨髓衰竭之间明显的矛盾在初诊时细胞减少型中更为明显。低增生性MDS（hMDS）似乎是一种独特的临床病理实体，约占所有MDS的15%。其发病机制被认为是免疫抑制机制导致的，并且有关于环孢素A（CsA）成功治疗的一些观察报道。本文介绍了一名年轻女性患者的病例，该患者通过骨髓活检确诊为hMDS - 难治性贫血（FAB和WHO分类），核型正常，免疫组化显示CD34(+)细胞稀少。她先接受雄激素治疗，随后使用CsA治疗数月，不久后发展为急性髓系白血病（M4），对低剂量每日口服美法仑有反应。这是关于MDS免疫抑制治疗期间发生此类事件的少数早期报道之一，并讨论了这种异常病程的可能解释。
