Duarte R F, Schmitz N, Servitje O, Sureda A
Department of Haematology, Institut Català d'Oncologia, Hospital Duran i Reynals, L'Hospitalet de Llobregat, Barcelona, Spain.
Bone Marrow Transplant. 2008 Apr;41(7):597-604. doi: 10.1038/sj.bmt.1705968. Epub 2008 Jan 7.
There is no standard of care for patients with advanced forms of mycosis fungoides, Sézary syndrome and other less common subtypes of primary cutaneous T-cell lymphoma. Expected median survival for such patients with conventional therapy is only 1-4 years. As a result of such dismal prognosis, alternative strategies based on autologous and allogeneic transplantation have been explored, and a relatively small number of case reports and small series communicated to date have provided evidence for the potential role of haematopoietic transplantation in these patients. High-dose radio-chemotherapy and autologous rescue has been shown to induce complete responses in the majority of patients. Disappointingly though, these responses were very short-lived in nearly all cases. On the contrary, the use of allogeneic transplantation has provided solid evidence for an allogeneic GVL effect in these malignancies. In fact, more than two-thirds of the allogeneic transplant recipients reported in the literature experienced long-term durable remissions of more than 3 years, which would appear superior to the expected median survival for such patients. This review summarizes the experience published to date in this setting and highlights main areas that would merit further investigation.
对于晚期蕈样肉芽肿、塞扎里综合征及其他较罕见的原发性皮肤T细胞淋巴瘤亚型患者，目前尚无标准治疗方案。采用传统疗法的这类患者的预期中位生存期仅为1至4年。鉴于预后如此不佳，人们探索了基于自体和异体移植的替代策略，迄今为止发表的相对少量的病例报告和小系列研究为造血移植在这些患者中的潜在作用提供了证据。高剂量放化疗及自体挽救治疗已显示可使大多数患者获得完全缓解。然而令人失望的是，几乎在所有病例中这些缓解都非常短暂。相反，异体移植的应用为这些恶性肿瘤中的异基因移植物抗白血病效应提供了确凿证据。事实上，文献报道的接受异体移植的患者中，超过三分之二经历了超过3年的长期持久缓解，这似乎优于这类患者的预期中位生存期。本综述总结了迄今为止在这方面发表的经验，并突出了值得进一步研究的主要领域。
