Anaplastic large-cell lymphoma (ALCL) accounts for approximately 10% of pediatric non-Hodgkin lymphoma (NHL). Previous experience from NHL-Berlin-Frankfurt-Münster (BFM) trials indicated that the short-pulse B-NHL-type treatment strategy may also be efficacious for ALCL. The purpose of this study was to test the efficacy of this protocol for treatment of childhood ALCL in a large prospective multicenter trial and to define risk factors. From April 1990 to March 1995, 89 patients younger than 18 years of age with newly diagnosed ALCL were enrolled in trial NHL-BFM 90. Immunophenotype was T-cell in 40 patients, B-cell in 5, null in 31, and not determined in 13. Stages were as follows: I, n = 8; II, n = 20; III, n = 55; IV, n = 6. Extranodal manifestations were as follows: mediastinum, n = 28; lung, n = 13; skin, n = 16; soft tissue, n = 13; bone, n = 14; central nervous system, n = 1; bone marrow, n = 5. After a cytoreductive prephase, treatment was stratified into 3 branches: patients in K1 (stage I and II resected) received three 5-day courses (methotrexate [MTX] 0.5 g/m(2), dexamethasone, oxazaphorins, etoposide, cytarabine, doxorubicin, and intrathecal therapy); patients in K2 (stage II nonresected and stage III) received 6 courses; patients in K3 (stage IV or multifocal bone disease) received 6 intensified courses including MTX 5 g/m(2), high-dose cytarabine/etoposide. The Kaplan-Meier estimate for a 5-year event-free survival was 76% +/- 5% (median follow-up, 5.6 years) for all patients and 100%, 73% +/- 6%, and 79% +/- 11% for K1, K2, and K3, respectively. Events were as follows: progression during therapy, n = 2; progression or relapse after therapy, n = 20; second malignancy, n = 1. It was concluded that short-pulse chemotherapy, stratified according to stage, is effective treatment for pediatric ALCL. B symptoms were associated with increased risk of failure. (Blood. 2001;97:3699-3706)
"This variant has become detectable only since the ALK1- immunohistochemistry became available for routine diagnostic analysis (Pittaluga et al, 1997; Pulford et al, 1997). The nine cases in which the diagnosis of non-anaplastic PTCL was changed to ALCL had an OS of 89% (data not shown), similar to survival rates described in contemporary ALCLstudies (Seidemann et al, 2001; Brugieres et al, 2009). In three patients the initial diagnosis of a PTCL was changed at review to a diagnosis of ALPS. "
[Show abstract][Hide abstract] ABSTRACT: Mature (peripheral) T-cell lymphoma (PTCL) other than anaplastic large cell lymphoma is a heterogeneous group of diseases and exceedingly rare in children and adolescents. Survival rates range between 46% and 85%. This study reports the disease characteristics, treatment and outcome of all patients with the diagnosis of mature TCL registered in the Berlin-Frankfurt-Munster non-Hodgkin lymphoma database between 1986 and 2012. All diagnoses were centrally reviewed and revised by clinico-pathological correlation according to the criteria of the current World Health Organization classification. Of the 69 patients originally registered as having PTCL, the diagnosis was confirmed in 38 of them. Most patients were treated with an anaplastic large cell lymphoma (ALCL)-like therapy regimen. Patients with PTCL-not otherwise specified comprised the largest group and showed a 5-year event-free survival rate of 61 ± 11%. Patients suffering from Natural Killer/T-cell- and hepatosplenic TCL had the poorest outcome. Our results suggest that the outcomes of children with mature TCL other than ALCL depend on the subtype and are worse than in all other paediatric lymphomas. The clinical experience presented in this largest study on paediatric mature TCL may serve as basis for future collaborative international prospective clinical trials.
British Journal of Haematology 11/2014; 168(6). DOI:10.1111/bjh.13216 · 4.71 Impact Factor
"Within the group of cardiac tumours, lymphomas represent o2%   and childhood cardiac lymphomas have only been reported in case reports   . However, primary cardiac childhood ALCL has not been described before and in the German paediatric NHL- BFM and the European ALCL99 studies, in which nearly 100% of all children with this disease are registered, no patient with cardiac ALCL was registered within the last 3 decades  . "
[Show abstract][Hide abstract] ABSTRACT: We report on an 8 year old boy with primary cardiac anaplastic large cell lymphoma (ALCL), in whom the diagnosis was challenging and who was treated with modified chemotherapy without radiation therapy according to the ALCL 99 study protocol . Two years and 4 months after completion of therapy the boy is in complete remission with normal cardiac function.
Leukemia Research Reports 05/2014; 3(2). DOI:10.1016/j.lrr.2014.05.001
"However, these studies were all single arm, which makes it difficult to draw a definitive conclusion about the superiority of transplantation. In addition, in many studies concerning conventional chemotherapy, the long-term OS rates ranged from 62% to 85% with a median follow-up time longer than 5 years, which was similar to the results of transplantation–. Therefore, it is necessary to compare conventional chemotherapy and transplantation in one setting to further evaluate the efficacy of transplantation on systemic ALCL. "
[Show abstract][Hide abstract] ABSTRACT: Anaplastic large-cell lymphoma (ALCL) is characterized by frequently presenting adverse factors at diagnosis. Many groups believed aggressive treatment strategies such as autologous stem cell transplantation brought survival benefit for ALCL patients. However, few compared these approaches with conventional chemotherapy to validate their superiority. Here, we report a study comparing the efficacy of peripheral blood stem cell transplantation (PBSCT) and conventional chemotherapy in the treatment of ALCL. A total of 64 patients with primary systemic ALCL were studied retrospectively. The median follow-up period was 51 months (range, 1-167 months). For 48 patients receiving conventional chemotherapy only, the 4-year event-free survival (EFS) and overall survival (OS) were 70.7% and 88.3%, respectively. Altogether, 16 patients received PBSCT, including 11 at first remission (CR1/PR1), 3 at second remission, and 2 for disease progression during first-line chemotherapy. The 4-year EFS and OS for patients transplanted at first remission were 81.8% and 90.9%, respectively. Compared with conventional chemotherapy, PBSCT did not show superiority either in EFS (P = 0.240) or in OS (P = 0.580) when applied at first remission. Univariate analysis showed that patients with B symptoms (P = 0.001), stage III/IV disease (P = 0.0083), bulky disease (P = 0.075), negative anaplastic lymphoma kinase (ALK) expression (P = 0.059), and age ≤ 60 years (P = 0.054) had lower EFS. Furthermore, PBSCT significantly improved EFS in patients with B symptoms (100% vs. 50.8%, P = 0.027) or bulky disease (100% vs. 52.8%, P = 0.045) when applied as an up-front strategy. Based on these results, we conclude that, for patients with specific adverse factors such as B symptoms or bulky disease, PBSCT was superior to conventional chemotherapy in terms of EFS.
Chinese journal of cancer 08/2012; 31(11). DOI:10.5732/cjc.011.10418 · 2.16 Impact Factor
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