Non‐anaplastic peripheral T‐cell lymphoma in children and adolescents – a retrospective analysis of the NHL ‐BFM study group
Udo KontnyIlske OschliesWilli WoessmannBirgit BurkhardtJasmin LisfeldJanina SalzburgAleš JandaAndishe AttarbaschiFelix NiggliMartin ZimmermannAlfred ReiterWolfram Klapper
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Abstract:
Summary 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.Keywords:
Anaplastic large-cell lymphoma
Peripheral T-cell lymphoma
Regimen
Not Otherwise Specified
T-Cell Lymphoma
B symptoms
Peripheral T-cell lymphoma
Not Otherwise Specified
T-Cell Lymphoma
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The T-cell lymphomas are characterized by marked differences in its global patterns of distribution. The epidemiology and classification of non-Hodgkin lymphoma (NHL) has undergone vast transformation over the past few decades. In an attempt to study the geographical distribution of NHL subtypes, the International NHL Classification Project was conducted in the late 1990s. The 2017 WHO classification broadly divides peripheral T-cell lymphomas (PTCL) into three categories based on their location. They are leukemic (disseminated), nodal, and extranodal. These categories are further subdivided based on morphology, immunohistochemistry, and clinical behavior. Examples of these lymphomas include PTCL not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphomas, anaplastic large-cell lymphoma (ALCL), anaplastic lymphoma kinase positive (ALK+), ALK negative (ALK–) ALCL, intestinal T-cell lymphoma, and breast implant-associated ALCL. This chapter discusses the epidemiological data for each of the individual subtype of PTCL.
Anaplastic large-cell lymphoma
Peripheral T-cell lymphoma
T-Cell Lymphoma
Large cell
Not Otherwise Specified
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Anaplastic large-cell lymphoma
Peripheral T-cell lymphoma
T-Cell Lymphoma
Not Otherwise Specified
Neoplasm
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Les lymphomes T périphériques (5 à 10 % de l’ensemble des lymphomes non hodgkiniens) appartiennent au groupe des lymphomes non hodgkiniens et plus particulièrement à celui des lymphoproliférations matures T/natural killer. Différentes entités de ces lymphomes sont décrites dans la classification 2008 de l’OMS avec des prévalences très variables. Leur diagnostic n’est pas toujours aisé et les mécanismes impliqués dans leur pathogenèse ne sont pas complètement élucidés. À l’exception du sous-type histologique lymphome anaplasique à grandes cellules ALK+, les lymphomes T périphériques se caractérisent par un pronostic sombre. Il semble primordial de définir de nouvelles stratégies thérapeutiques, qui pourraient être fondées sur l’administration de thérapies ciblées. Le développement de nouvelles techniques de séquençage à haut débit offre désormais la possibilité d’identifier des événements génétiques possiblement importants dans le développement des tumeurs. Ces analyses, appliquées aux échantillons tumoraux de lymphomes T périphériques, ont ainsi permis de détecter des anomalies moléculaires spécifiques de certains sous-types, qui pourraient être intégrées dans la future classification et constituer également de nouvelles cibles thérapeutiques. Cette revue a pour but de faire un état des lieux des connaissances actuelles sur les évènements moléculaires à l’origine des lymphomes T périphériques ou survenant pendant leur histoire naturelle. Elle comprend deux parties, l’une (publiée dans ce numéro) consacrée aux trois entités les plus fréquentes : le lymphome T angio-immunoblastique, le lymphome T périphérique non spécifié et le lymphome anaplasique à grandes cellules ; l’autre (à paraître dans le numéro de novembre 2015)1 portera sur des sous-types plus rares et de mauvais pronostic : le lymphome NK/T extra-ganglionnaire de type nasal, la leucémie/lymphome T de l’adulte HTLV1+ et le lymphome T associé à une entéropathie. Les lymphoproliférations T de présentation essentiellement leucémique (leucémie à cellules natural killer, leucémies à grands lymphocytes à grains, leucémie prolymphocytaire T), les lymphomes T primitifs cutanés (syndrome de Sézary, mycosis fungoïde) et les entités de lymphomes T périphériques dont la prévalence est inférieure à 5 % (lymphome T hépatosplénique, lymphome T sous-cutané de type panniculite) ne seront pas traités ici.
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AbstractThe purpose of this study was to better define the clinical features and natural history of peripheral T-cell lymphomas (PTCL) entities included in the Revised European American lymphoma (REAL) classification. Cases of PTCL were retrieved from the records of the Department of Pathology and classified according to the REAL classification. In addition, cases of anaplastic large cell lymphoma (ALCL) were divided into classical, small cell, and primary cutaneous subtypes, and immunostaining for the anaplastic large-cell kinase (ALK) protein was performed on all cases of ALCL. Clinical features, response to therapy and survival were abstracted. Ninety-two cases of PTCL with adequate clinical information were retrieved. There were 40 cases of ALCL (30 classical, 7 small cell variant, 3 primary cutaneous), 28 PTCL, unspecified, 13 angioimmunoblastic T-cell lymphoma and 11 with other entities. The patients had a median age of 48 years with a range of 6-84 and had an estimated overall survival (OS) of 49% and progression-free survival (PFS) of 22% at 5 years. The International Prognostic Index (IPI) was a significant prognostic factor for both progression-free and OS. Histology was a significant predictor of PFS with anaplastic large cell having the best prognosis. ALK expression was not associated with an improved progression-free or overall-survival in patients with systemic T-cell ALCL. In conclusion, the REAL classification describes distinct PTCL entities. The IPI is the most important predictor of progression-free and OS in patients with PTCL. ALK expression may not provide prognostic information for systemic ALCL.KeywordsPeripheral T-cell LymphomasClinical FeaturesPrognostic FactorsReal ClassificationMalignant Lymphoma
Peripheral T-cell lymphoma
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Using pathological and clinical review, to identify all cases diagnosed as peripheral T cell and natural killer (NK) T cell lymphoma over 10 years from one metropolitan Australian hospital.Subtyping was performed using World Health Organization (WHO) 2008 criteria and a comprehensive immunohistochemical panel. Clinical data including follow-up were obtained. There were 47 cases, including 11 peripheral T cell lymphomas, not otherwise specified (NOS), nine extranodal NK T cell lymphomas, nasal type (eight nasal), eight primary cutaneous anaplastic large cell lymphomas, seven angioimmunoblastic T cell lymphomas, three anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphomas, four ALK-negative anaplastic large cell lymphomas, three enteropathic T cell lymphomas and two subcutaneous panniculitis-like T cell lymphomas. Follow-up of 46 of 47 cases (median time 45 months) revealed that 50% (23 of 46) of patients died. Five-year survival rates were: peripheral T cell lymphoma, NOS 39%; angioimmunoblastic T cell, 43%; nasal NK T 67%; ALK-negative anaplastic large cell lymphoma 67% (at 2 years); ALK(+) anaplastic large cell lymphoma 33%; subcutaneous panniculitis-like T cell lymphomas 100%; primary cutaneous anaplastic large cell lymphoma 86%; and enteropathic T cell lymphoma 33% (at 1 year). One patient with Lennert lymphoma suffered four late cutaneous relapses.This first Australian clinicopathological series of peripheral T cell and NK T cell lymphoma shows epidemiological and survival data similar to those for Europe and North America.
Anaplastic large-cell lymphoma
T-Cell Lymphoma
Large cell
Peripheral T-cell lymphoma
Not Otherwise Specified
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The peripheral T-cell lymphomas represent about 15% to 20% of non-Hodgkin lymphomas and are marked by clinical and pathologic heterogeneity. The most common T-cell entities include peripheral T-cell lymphoma, not otherwise specified, angioimmunoblastic T-cell lymphoma, and anaplastic large cell lymphoma anaplastic lymphoma kinase-negative, which account for approximately 60% of T-cell lymphoma cases. Because of the rarity of T-cell lymphomas and lack of randomized prospective studies, treatment for these diseases is not well defined. Current treatment strategies draw from data from phase II studies, retrospective analyses, and personal experience. For fit patients who can tolerate treatment with curative intent, we treat peripheral T-cell lymphoma, not otherwise specified, angioimmunoblastic T-cell lymphoma, and anaplastic large cell lymphoma anaplastic lymphoma kinase-negative similarly with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)-based induction therapy followed by consolidation with autologous stem cell transplant. Given the marked differences in histology, biology, and clinical presentation for these diseases, it is likely that they should be approached differently. Furthermore, prognostic factors and degree of chemosensitivity as measured by FDG-PET (fluorodeoxyglucose positron emission tomography) should likely be used to guide patients along different treatment pathways. We have a long way to go toward perfecting the treatment for T-cell lymphoma. We believe that a uniform treatment approach for patients with aggressive T-cell lymphoma is not appropriate; however, we do not yet have enough data to support an individualized approach to treatment. Clinical and biologic prognostic factors, degree of chemosensitivity as measured by FDG-PET, and histology should all likely have a role in directing patients along different treatment pathways, but prospective studies are needed to confirm this.
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Aggressive lymphoma
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