Extramedullary Disease in Adult Acute Myeloid Leukemia Is Common but Lacks Independent Significance: Analysis of Patients in ECOG-ACRIN Cancer Research Group Trials, 1980-2008

Extramedullary Disease in Adult Acute Myeloid Leukemia Is Common but Lacks Independent Significance: Analysis of Patients in ECOG-ACRIN Cancer Research Group Trials, 1980-2008
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DOI:
10.1200/jco.2016.67.5892
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发表时间:
2016-10-10
影响因子:
45.3
通讯作者:
Tallman, Martin S.
Tallman, Martin S.
中科院分区:
医学1区
文献类型:
--
作者:
Ganzel, Chezi;Manola, Judith;Tallman, Martin S.

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目的急性髓系白血病(AML)患者诊断时存在髓外病变(EMD)已被认识几十年。本文报道的是来自AML患者的大型研究的结果,这些患者在连续的ECOG-ACRIN癌症研究组一线临床试验中接受治疗,试图确定EMD的发病率和临床意义。结果在3,522例入选的患者中,282例被排除,包括急性早幼粒细胞白血病、诊断错误或基线时未对EMD进行充分评估的患者。EMD的总发生率为23.7%。累及部位为:淋巴结(11.5%)、脾脏(7.3%)、肝脏(5.3%)、皮肤(4.5%)、牙龈(4.4%)和CNS(1.1%)。大部分患者(65.3%)仅有1个EMD部位,20.9%有2个,9.5%有3个,3.4%有4个,中位生存期为1.035年。在单变量分析中,存在任何EMD(P = .005)、皮肤受累(P = .002)、脾脏(P < .001)和肝脏(P < .001),而不是CNS(P = .34)、淋巴结受累(P = .94)和牙龈肥大(P = .24),与总生存期较短相关。相反,在多变量分析,调整已知的预后因素,如细胞遗传学风险和白细胞计数,无论是EMD的存在,也不是EMD的具体网站的数量是independentprojective.ConclusionThis大型研究表明,EMD在任何网站是常见的,但不是一个独立的预后因素。EMD患者的治疗决定应根据公认的AML预后因素做出,无论是否存在EMD。
PurposeExtramedullary disease (EMD) at diagnosis in patients with acute myeloid leukemia (AML) has been recognized for decades. Reported herein are results from a large study of patients with AML who were treated in consecutive ECOG-ACRIN Cancer Research Group frontline clinical trials in an attempt to define the incidence and clinical implications of EMD.MethodsPatients with newly diagnosed AML, age 15 years and older, who were treated in 11 clinical trials, were studied to identify EMD, as defined by physical examination, laboratory findings, and imaging results.ResultsOf the 3,522 patients enrolled, 282 were excluded, including patients with acute promyelocytic leukemia, incorrect diagnosis, or no adequate assessment of EMD at baseline. The overall incidence of EMD was 23.7%. The sites involved were: lymph nodes (11.5%), spleen (7.3%), liver (5.3%), skin (4.5%), gingiva (4.4%), and CNS (1.1%). Most patients (65.3%) had only one site of EMD, 20.9% had two sites, 9.5% had three sites, and 3.4% had four sites.The median overall survival was 1.035 years. In univariable analysis, the presence of any EMD (P = .005), skin involvement (P = .002), spleen (P < .001), and liver (P < .001), but not CNS (P = .34), nodal involvement (P = .94), and gingival hypertrophy (P = .24), was associated with a shorter overall survival. In contrast, in multivariable analysis, adjusted for known prognostic factors such as cytogenetic risk and WBC count, neither the presence of EMD nor the number of specific sites of EMD were independently prognostic.ConclusionThis large study demonstrates that EMD at any site is common but is not an independent prognostic factor. Treatment decisions for patients with EMD should be made on the basis of recognized AML prognostic factors, irrespective of the presence of EMD.