Poiré Xavier, Labopin Myriam, Maertens Johan, Yakoub-Agha Ibrahim, Blaise Didier, Ifrah Norbert, Socié Gérard, Gedde-Dhal Tobias, Schaap Nicolaas, Cornelissen Jan J, Vigouroux Stéphane, Sanz Jaime, Michaux Lucienne, Esteve Jordi, Mohty Mohamad, Nagler Arnon
Section of Hematology, Department of Medicine, Cliniques Universitaires Saint-Luc, 10, avenue Hippocrate, 1200, Brussels, Belgium.
Acute Leukemia Working Party of the EBMT office, Paris, France.
J Hematol Oncol. 2017 Jan 18;10(1):20. doi: 10.1186/s13045-017-0393-3.
Acute myeloid leukaemia (AML) with 17p abnormalities (abn(17p)) carries a very poor prognosis due to high refractoriness to conventional chemotherapy, and allogeneic stem cell transplantation (allo-SCT) appears as the only potential curative option.
To address outcomes after allo-SCT in patients with abn(17p), we retrospectively analysed de novo or secondary AML undergoing SCT between 2000 and 2013 from the EBMT registry.
One hundred thirty-nine patients with confirmed abn(17p) have been selected. At the time of transplant, one hundred twenty-five were in first remission (CR1). Median age was 54 years old. Abn(17p) was associated with a monosomal karyotype in 83% of patients, complex karyotype in 91%, monosomy 5 or 5q deletion (-5/5q-) in 55%, monosomy 7 (-7) in 39% and both -5/5q and -7 in 27%. Seventy-three patients (59%) had a reduced-intensity conditioning regimen. The 2-year overall survival (OS) and leukaemia-free survival (LFS) were 28 and 24%, respectively. The 2-year non-relapse mortality (NRM) was 15%, and 2-year relapse incidence (RI) was 61%. The cumulative incidence of grade II to IV acute graft-versus-host disease (GvHD) was 24% and that of chronic GvHD was 21%. In multivariate analysis, the presence of a -5/5q- in addition to abn(17p) was significantly and independently associated with worse OS, LFS and higher RI. Age and donor types did not correlate with outcome. Conditioning intensity was not statistically associated with OS, LFS and NRM when adjusted for patients' age.
In contrast to the dismal prognosis reported for AML patients harbouring abn(17p) undergoing conventional chemotherapy, allogeneic SCT provides responses in about 25% of those patients transplanted in CR1.
伴有17号染色体短臂异常(abn(17p))的急性髓系白血病(AML)由于对传统化疗高度耐药,预后极差,而异基因干细胞移植(allo-SCT)似乎是唯一潜在的治愈选择。
为了探讨allo-SCT治疗abn(17p)患者后的结局,我们对2000年至2013年间从欧洲血液与骨髓移植登记处接受SCT的初发或继发AML患者进行了回顾性分析。
共入选139例确诊为abn(17p)的患者。移植时,125例处于首次缓解期(CR1)。中位年龄为54岁。83%的患者abn(17p)与单体核型相关,91%与复杂核型相关,55%与5号染色体单体或5号染色体长臂缺失(-5/5q-)相关,39%与7号染色体单体(-7)相关,27%与-5/5q-和-7均相关。73例患者(59%)采用了减低强度预处理方案。2年总生存率(OS)和无白血病生存率(LFS)分别为28%和24%。2年非复发死亡率(NRM)为15%,2年复发率(RI)为61%。II至IV级急性移植物抗宿主病(GvHD)的累积发生率为24%,慢性GvHD的累积发生率为21%。多因素分析显示,除abn(17p)外,存在-5/5q-与较差的OS、LFS及较高的RI显著且独立相关。年龄和供体类型与结局无关。在根据患者年龄进行调整后,预处理强度与OS、LFS和NRM无统计学关联。
与报道的接受传统化疗的伴有abn(17p)的AML患者的预后不佳相反,异基因SCT使约25%处于CR1期接受移植患者获得缓解。