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阴性骨髓增殖性肿瘤转化为加速期或白血病期的转归

Outcome of Ph negative myeloproliferative neoplasms transforming to accelerated or leukemic phase.

作者信息

Mollard Lise-Marie, Chauveau Aurélie, Boyer-Perrard Françoise, Douet-Guilbert Nathalie, Houot Roch, Quintin-Roué Isabelle, Couturier Marie-Anne, Dagorne Anaig, Malou Mohamed, Le Calloch Ronan, Luycx Odile, Thepot Sylvain, Hunault Mathilde, Guillerm Gaelle, Berthou Christian, Ugo Valérie, Lippert Éric, Ianotto Jean-Christophe

机构信息

a Service d'Hématologie Clinique, Institut de Cancéro-Hématologie, CHRU de Brest , Brest , France.

b Laboratoire d'Hématologie, CHRU de Brest , Brest , France.

出版信息

Leuk Lymphoma. 2018 Dec;59(12):2812-2820. doi: 10.1080/10428194.2018.1441408. Epub 2018 Apr 4.

Abstract

Myeloproliferative neoplasms (MPN) are chronic disorders that can sometimes evolve into accelerated or leukemic phases. We retrospectively identified 122 patients with such blastic phases. The overall median survival was four months: 10.2 months for patients treated with intensive treatments compared to three months for best supportive care (p = .005). Azacytidine, intensive chemotherapies, or allogeneic stem cell transplantation gave the highest median survivals with 9, 10.2, and 19.4 months, respectively. Accelerated phases (AP) had a longer median survival compared to acute leukemia (4.8 months vs. 3.1 months; p = .02). In this retrospective and observational study, we observe that the longest survivals are seen in patients eligible for intensive treatments. Azacytidine shows interesting results in patients non-fit for intensive chemotherapy. Supportive care should probably be restricted to elderly patients and those with unfavorable karyotype. An early diagnosis of AP could also result in a better survival rate.

摘要

骨髓增殖性肿瘤(MPN)是一种慢性疾病,有时会发展为加速期或白血病期。我们回顾性地确定了122例处于此类原始细胞期的患者。总体中位生存期为4个月:接受强化治疗的患者为10.2个月,而最佳支持治疗的患者为3个月(p = 0.005)。阿扎胞苷、强化化疗或异基因干细胞移植的中位生存期最长,分别为9个月、10.2个月和19.4个月。加速期(AP)的中位生存期比急性白血病更长(4.8个月对3.1个月;p = 0.02)。在这项回顾性观察研究中,我们观察到,适合强化治疗的患者生存期最长。阿扎胞苷在不适合强化化疗的患者中显示出有趣的结果。支持治疗可能应限于老年患者和核型不良的患者。AP的早期诊断也可能导致更高的生存率。

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