Allogeneic stem cell transplantation for myeloproliferative neoplasm in blast phase
Allogeneic stem cell transplantation for myeloproliferative neoplasm in blast phase
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DOI：
10.1016/j.leukres.2012.04.021
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发表时间：
2012-09-01
影响因子：
2.7
通讯作者：
Mesa, Ruben A.
中科院分区：
文献类型：
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作者：
Cherington, Chad;Slack, James L.;Mesa, Ruben A.
费城阴性骨髓增生性肿瘤（MPN）患者演变为急性髓细胞白血病（AML）或急变期（MPN-BP）的预后极差。尽管异基因干细胞移植（allo-SCT）被认为具有潜在的治愈性，但很少有患者报告接受了用于MPN-BP的allo-SCT;因此，成功率仍然未知。在一项回顾性研究中，我们确定了13例MPN在中位9年（范围5个月至30年）后转化为急变期的患者; 8例（中位年龄55岁）在6个月内继续接受allo-SCT。诱导化疗清除了60%（6/10）的血液/骨髓原始细胞（2例拒绝治疗，1例早期死亡）。在allo-SCT时，5/8例患者的白血病完全缓解（CR）或已恢复至MPN慢性期（CP），2例有残留的血母细胞，1例难治性骨髓母细胞>5%。在随访时（中位20.3个月），6例患者的白血病/MPN均存活于CR中。所有5例在allo-SCT前的CR/CP患者在缓解期仍然存活，而2/3例持续性血液/骨髓原始细胞复发并死亡。我们的结论是，MPN-BP可以治愈的allo-SCT在一个显着的百分比的患者，但足够的白血病清除allo-SCT前提供了一个最佳的结果。(c)2012爱思唯尔有限公司保留所有权利。
The prognosis for patients with Philadelphia-negative myeloproliferative neoplasms (MPN) who evolve into acute myeloid leukemia (AML) or blast phase (MPN-BP) is extremely poor. Although allogeneic stem cell transplantation (allo-SCT) is considered potentially curative, very few patients have been reported who have undergone allo-SCT for MPN-BP; therefore the success rate remains unknown. In a retrospective review, we identified 13 patients with an MPN transformation to blast phase after a median 9 years (range 5 months to 30 years); 8 (median age 55) continued to allo-SCT within 6 months. Induction chemotherapy cleared blood/marrow blasts in 60% (6/10) (2 declined therapy, 1 had early death). At the time of allo-SCT, 5/8 patients were in complete remission (CR) of their leukemia or had returned to MPN chronic phase (CP), 2 had residual blood blasts and 1 was refractory with >5% marrow blasts. At follow-up (median 20.3 months), 6 patients are alive in CR of both their leukemia/MPN. All 5 patients in CR/CP at pre-allo-SCT remain alive in remission, while 2/3 with persistent blood/marrow blasts relapsed and expired. We conclude that MPN-BP can be cured by allo-SCT in a significant percentage of patients, but that adequate leukemic clearance prior to allo-SCT offers an optimal outcome. (c) 2012 Elsevier Ltd. All rights reserved.