Article

Prognostic factors in childhood anaplastic large cell lymphoma: results of a large European intergroup study.

Biostatistics and Epidemiology Unit, Institut Gustave-Roussy, Villejuif, France.
Blood (Impact Factor: 9.06). 02/2008; 111(3):1560-6. DOI:10.1182/blood-2007-07-100958
Source: PubMed

ABSTRACT To study prognostic factors of progression/relapse, data concerning 225 children enrolled between 1987 and 1997 in Berlin-Frankfurt-Münster, Société Française d'Oncologie Pédiatrique and United Kingdom Children's Cancer Study Group prospective studies for the treatment of anaplastic large cell lymphoma (ALCL) were merged. Median follow-up was 9.3 years. Five-year overall survival and event-free survival of the whole population was 81% (95% confidence interval, 76%-86%) and 69% (63%-74%), respectively. B symptoms, mediastinal involvement, skin lesions, visceral involvement, St Jude stage 3-4, Ann Arbor stage 3-4, and elevated lactate dehydrogenase increased the risk of progression/relapse in the univariate analysis. In the multivariate analysis, 3 factors remained significant: mediastinal involvement (relative risk [RR] = 2.1 [1.2-3.5]), visceral involvement defined as lung, liver, or spleen involvement (RR = 2.1 [1.3-3.6]), and skin lesions (RR = 1.9 [1.1-3.2]). Five-year progression-free survival (PFS) of the 81 patients with none of these risk factors was 89% [82%-96%], contrasting with a 5-year PFS of 61% [53%-69%] in the 144 patients with at least 1 risk factor (RR = 4.4 [2.2-8.9; P < .001). In conclusion, 3 factors associated with an increased risk of failure in childhood ALCL have been defined: mediastinal involvement, visceral involvement, and skin lesions.

0 0
 · 
1 Bookmark
 · 
77 Views
  • [show abstract] [hide abstract]
    ABSTRACT: The New York protocol, primarily developed to treat children with high-risk acute lymphoblastic leukemia (ALL), is characterized by early intensive chemotherapy followed by less intensive pulse chemotherapy during maintenance. This study was performed to evaluate the efficacy of this protocol in children with non-Hodgkin lymphoma (NHL), irrespective of histopathologic subtype. From January 1996 to December 2011, 146 newly diagnosed children and adolescents with NHL were treated with the modified New York protocol. Treatment duration was determined according to the stage. The 5-year failure-free survival (FFS), event-free survival (EFS), and overall survival (OS) rates were 86.7 ± 2.9%, 79.1 ± 3.5%, and 84.7 ± 3.1%, respectively. The 5-year FFS for patients with mature B-cell lymphoma, T-cell and NK-cell lymphoma (T/NK-cell lymphoma), and lymphoblastic lymphoma were 95.4 ± 2.6%, 76.1 ± 7.0%, and 82.1 ± 6.6%, respectively. In multivariate analysis, T/NK-cell lymphoma and non-complete response (non-CR) at the end of induction chemotherapy were associated with a significant increase in treatment failure rate (relative risk [RR], 4.5, P = 0.03, and RR, 5.0, P = 0.002). The protocol appears to be efficacious in the treatment of children and adolescents with NHL, irrespective of histopathologic subtype. Achievement of CR after intensive induction chemotherapy was an important prognostic factor. Early response to treatment may be used to stratify risk groups and modify therapy in children with NHL. Pediatr Blood Cancer © 2013 Wiley Periodicals, Inc.
    Pediatric Blood & Cancer 07/2013; · 2.35 Impact Factor
  • [show abstract] [hide abstract]
    ABSTRACT: Pediatric non-Hodgkin's lymphomas (NHLs) are a heterogeneous group of malignancies with distinct clinical, pathological, immunological and genetic characteristics. More than 90% of pediatric NHLs belong to one of three major histological subtypes: mature B-cell neoplasms, lymphoblastic lymphomas and anaplastic large-cell lymphomas. The recognition that different subtypes require different treatment regimens resulted in therapeutic strategies leading to over 80% of patients being cured. On the other hand, patients with resistant or relapsed disease have a poor prognosis. Prognostic biomarkers have not yet been identified for all pediatric NHLs and, although some are very important for diagnosis and prognosis, others may be of questionable value. Discovery of new biomarkers suitable for clinical application may aid the diagnosis and classification of lymphomas, which should, in turn, lead to better patient stratification. Consequent development of new treatment and follow-up approaches should lead to more efficient and less toxic treatment in children with NHL.
    Biomarkers in Medicine 10/2013; 7(5):791-801. · 3.22 Impact Factor
  • [show abstract] [hide abstract]
    ABSTRACT: Anaplastic lymphoma kinase positive anaplastic large T-cell lymphoma is characterized by morphological variability. Morphological variants (non-common sutype) are associated with a poor outcome. They display abundant reactive bystander cells admixed with the lymphoma cells. So far, the difficulty distinguishing lymphoma cells from bystander cells by visual inspection has prevented detailed and reliable immunophenotypic analysis using conventional immunohistochemistry. To overcome these limitations, we analyzed 124 cases of pediatric anaplastic lymphoma kinase positive anaplastic large cell lymphoma treated within clinical trials using immunofluorescence multi-staining and digital image analysis combining antibodies against anaplastic lymphoma kinase to specifically identify lymphoma cells with antibodies against CD30, CD3, CD5, CD8, Ki67 and phosphorylated STAT3. Non-common type anaplastic lymphoma kinase positive anaplastic large cell lymphoma express CD8 more frequently than common type anaplastic lymphoma kinase positive anaplastic large cell lymphoma (35.4% and 5.6%, respectively, p=0.0002). CD8 expression was associated with a poorer outcome. Importantly, in a multivariate-analysis including clinical risk factors, histological subtype and CD8 expression, CD8-positivity proved to be an independent prognostic predictor of worse outcome (hazard ratio for survival 3.38, p=0.042).
    Haematologica 05/2013; · 5.94 Impact Factor

Full-text

View
0 Downloads