Putti M C, Rondelli R, Cocito M G, Aricó M, Sainati L, Conter V, Guglielmi C, Cantú-Rajnoldi A, Consolini R, Pession A, Zanesco L, Masera G, Biondi A, Basso G
Dipartimento di Pediatria, Universitá di Padova, Padova; III Clinica Pediatrica, Universitá di Bologna, Bologna, Italy.
Blood. 1998 Aug 1;92(3):795-801.
The importance of coexpression of myeloid antigens in childhood acute lymphoblastic leukemia (ALL) has long been debated; results are conflicting. We studied children with ALL treated at Italian Association for Pediatric Hematology-Oncology (AIEOP) institutions over 6 years with Berlin-Frankfurt-Muenster (BFM)-based protocols and have analyzed the incidence of coexpression of six MyAg (CD11b, CD13, CD14, CD15, CD33, CD65w) to determine its prognostic impact. Criteria for MyAg coexpression (MyAg+ALL) included positivity to one or more MyAg on at least 20% of blasts and confirmation of coexpression at double-fluorescence analysis. A total of 291 of 908 cases were MyAg+ALL (32%). Incidence was similar in B-ALL and T-ALL; among common, pre-B, and pre-pre-B-ALL. CD13 and CD33 were most common. Patients with MyAg+ALL had presenting features similar to MyAg-ALL. They entered standard or intermediate risk protocols more frequently and had better prednisone response, but similar complete remission rates. Six-year event-free survival (EFS) was 69.0% in 291 MyAg+ALL cases and 65.3% in 617 MyAg-ALL cases, without significant difference. Cases expressing two or more MyAg presented similar clinical features and treatment response. MyAg+ALL had worse EFS only in infants (0% v 47%) (P = .01). Therefore, in this series of homogeneously diagnosed and treated ALL, coexpression of MyAg was not associated with prognostic significance, without relevance for clinical purposes or for patient stratification, except for infants.
髓系抗原共表达在儿童急性淋巴细胞白血病(ALL)中的重要性长期以来一直存在争议;结果相互矛盾。我们研究了在意大利儿童血液肿瘤协会(AIEOP)机构接受治疗超过6年的ALL患儿,这些患儿采用基于柏林-法兰克福-明斯特(BFM)的方案,并分析了六种髓系抗原(MyAg,即CD11b、CD13、CD14、CD15、CD33、CD65w)共表达的发生率,以确定其预后影响。MyAg共表达(MyAg+ALL)的标准包括至少20%的原始细胞对一种或多种MyAg呈阳性,并在双荧光分析中确认共表达。908例病例中共有291例为MyAg+ALL(32%)。B-ALL和T-ALL中的发生率相似;在普通型、前B细胞型和前前B细胞型ALL中也是如此。CD13和CD33最为常见。MyAg+ALL患者的临床表现与MyAg-ALL相似。他们更频繁地进入标准或中危方案,对泼尼松的反应更好,但完全缓解率相似。291例MyAg+ALL病例的6年无事件生存率(EFS)为69.0%,617例MyAg-ALL病例为65.3%,无显著差异。表达两种或更多MyAg的病例具有相似的临床特征和治疗反应。MyAg+ALL仅在婴儿中EFS较差(0%对47%)(P = 0.01)。因此,在这一系列诊断和治疗均一致的ALL中,MyAg共表达与预后意义无关,对临床目的或患者分层无相关性,但婴儿除外。