Hunter D Niehus, Jean Sabile, Richard T Maziarz, Gabrielle Meyers, Rachel Cook, Arpita P Gandhi, Jennifer N Saultz, Shauna Rakshe, Andy Kaempf, Theodore Braun, Yazan Migdady
{"title":"异基因造血细胞移植后慢性粒细胞白血病（CMML）增生异常亚型的存活率高于增生异常亚型：一个三级中心的经验和文献综述。","authors":"Hunter D Niehus, Jean Sabile, Richard T Maziarz, Gabrielle Meyers, Rachel Cook, Arpita P Gandhi, Jennifer N Saultz, Shauna Rakshe, Andy Kaempf, Theodore Braun, Yazan Migdady","doi":"10.1159/000539880","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>CMML is a rare neoplasm with overlapping myelodysplastic and myeloproliferative features whose only potential cure is allogeneic hematopoietic cell transplantation (allo-HCT).</p><p><strong>Methods: </strong>This retrospective study examined 27 CMML patients with high-risk clinical features who underwent first allo-HCT at our institution between 2004 and 2022.</p><p><strong>Results: </strong>Nineteen patients were diagnosed with the proliferative subtype (CMML-MPN) and 8 with the dysplastic subtype (CMML-MDS). Median OS was 15 months post-HCT (95% CI: 5-71); OS at 1, 3, and 5 years was 52%, 35%, and 35%, respectively. Compared to those with CMML-MPN, patients with CMML-MDS had longer OS (median, 8.6 vs. 0.9 years; p = 0.025), RFS (4.4 vs. 0.5 years; p = 0.021), and GVHD-free, relapse-free survival (GRFS, 9.4 vs. 3.4 months; p = 0.033) as well as lower 1-year NRM (13 vs. 47%; p = 0.043), with the statistical significance of this CMML subtype effect maintained in multivariable models. High-risk cytogenetics were associated with shorter GRFS in the univariable (median, 3.1 vs. 6.2 months; p = 0.013) and multivariable (HR = 4.88; p = 0.006) settings.</p><p><strong>Conclusions: </strong>Patients who underwent transplant for CMML-MDS experienced substantially better outcomes than those transplanted for CMML-MPN. Future studies are needed for transplantation optimization in CMML, especially CMML-MPN.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-10"},"PeriodicalIF":1.7000,"publicationDate":"2024-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Enhanced Survival of Chronic Myelomonocytic Leukemia-Dysplastic over Proliferative Subtype after Allogeneic Hematopoietic Cell Transplant: A Tertiary Center Experience and Literature Review.\",\"authors\":\"Hunter D Niehus, Jean Sabile, Richard T Maziarz, Gabrielle Meyers, Rachel Cook, Arpita P Gandhi, Jennifer N Saultz, Shauna Rakshe, Andy Kaempf, Theodore Braun, Yazan Migdady\",\"doi\":\"10.1159/000539880\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>CMML is a rare neoplasm with overlapping myelodysplastic and myeloproliferative features whose only potential cure is allogeneic hematopoietic cell transplantation (allo-HCT).</p><p><strong>Methods: </strong>This retrospective study examined 27 CMML patients with high-risk clinical features who underwent first allo-HCT at our institution between 2004 and 2022.</p><p><strong>Results: </strong>Nineteen patients were diagnosed with the proliferative subtype (CMML-MPN) and 8 with the dysplastic subtype (CMML-MDS). Median OS was 15 months post-HCT (95% CI: 5-71); OS at 1, 3, and 5 years was 52%, 35%, and 35%, respectively. Compared to those with CMML-MPN, patients with CMML-MDS had longer OS (median, 8.6 vs. 0.9 years; p = 0.025), RFS (4.4 vs. 0.5 years; p = 0.021), and GVHD-free, relapse-free survival (GRFS, 9.4 vs. 3.4 months; p = 0.033) as well as lower 1-year NRM (13 vs. 47%; p = 0.043), with the statistical significance of this CMML subtype effect maintained in multivariable models. High-risk cytogenetics were associated with shorter GRFS in the univariable (median, 3.1 vs. 6.2 months; p = 0.013) and multivariable (HR = 4.88; p = 0.006) settings.</p><p><strong>Conclusions: </strong>Patients who underwent transplant for CMML-MDS experienced substantially better outcomes than those transplanted for CMML-MPN. 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引用次数: 0
摘要
导言 CMML是一种罕见的肿瘤，具有骨髓增生异常和骨髓增生性重叠的特征，唯一可能治愈的方法是异基因造血细胞移植（allo-HCT）。方法 这项回顾性研究考察了 2004 年至 2022 年期间在我院接受首次异基因造血干细胞移植的 27 例具有高危临床特征的 CMML 患者。结果 19例患者被诊断为增殖亚型（CMML-MPN），8例为发育不良亚型（CMML-MDS）。中位OS为HCT后15个月（95% CI：5-71）；1年、3年和5年的OS分别为52%、35%和35%。与CMML-MPN患者相比，CMML-MDS患者的OS（中位数，8.6年 vs 0.9年；P=0.025）、RFS（4.4年 vs 0.5年；P=0.021）、无GVHD、无复发生存期（GRFS，9.4个月 vs 3.4个月；P=0.033）更长，1年NRM更低（13% vs 47%；P=0.043），这种CMML亚型效应在多变量模型中仍具有统计学意义。在单变量（中位 3.1 个月 vs 6.2 个月；P=0.013）和多变量（HR=4.88；P=0.006）设置中，高风险细胞遗传学与较短的 GRFS 相关。结论 因CMML-MDS而接受移植的患者的预后大大优于因CMML-MPN而接受移植的患者。未来需要对CMML，尤其是CMML-MPN的移植优化进行研究。
Enhanced Survival of Chronic Myelomonocytic Leukemia-Dysplastic over Proliferative Subtype after Allogeneic Hematopoietic Cell Transplant: A Tertiary Center Experience and Literature Review.
Introduction: CMML is a rare neoplasm with overlapping myelodysplastic and myeloproliferative features whose only potential cure is allogeneic hematopoietic cell transplantation (allo-HCT).
Methods: This retrospective study examined 27 CMML patients with high-risk clinical features who underwent first allo-HCT at our institution between 2004 and 2022.
Results: Nineteen patients were diagnosed with the proliferative subtype (CMML-MPN) and 8 with the dysplastic subtype (CMML-MDS). Median OS was 15 months post-HCT (95% CI: 5-71); OS at 1, 3, and 5 years was 52%, 35%, and 35%, respectively. Compared to those with CMML-MPN, patients with CMML-MDS had longer OS (median, 8.6 vs. 0.9 years; p = 0.025), RFS (4.4 vs. 0.5 years; p = 0.021), and GVHD-free, relapse-free survival (GRFS, 9.4 vs. 3.4 months; p = 0.033) as well as lower 1-year NRM (13 vs. 47%; p = 0.043), with the statistical significance of this CMML subtype effect maintained in multivariable models. High-risk cytogenetics were associated with shorter GRFS in the univariable (median, 3.1 vs. 6.2 months; p = 0.013) and multivariable (HR = 4.88; p = 0.006) settings.
Conclusions: Patients who underwent transplant for CMML-MDS experienced substantially better outcomes than those transplanted for CMML-MPN. Future studies are needed for transplantation optimization in CMML, especially CMML-MPN.
期刊介绍：
''Acta Haematologica'' is a well-established and internationally recognized clinically-oriented journal featuring balanced, wide-ranging coverage of current hematology research. A wealth of information on such problems as anemia, leukemia, lymphoma, multiple myeloma, hereditary disorders, blood coagulation, growth factors, hematopoiesis and differentiation is contained in first-rate basic and clinical papers some of which are accompanied by editorial comments by eminent experts. These are supplemented by short state-of-the-art communications, reviews and correspondence as well as occasional special issues devoted to ‘hot topics’ in hematology. These will keep the practicing hematologist well informed of the new developments in the field.
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