Peripheral T-cell lymphomas (PTCL) have been generally reported to have a worse prognosis than B-cell lymphomas (BCL). Because of their heterogeneity and scarcity, the outcomes of the different histological subtypes have not been compared. From October 1987 to March 1993, 1,883 patients with diffuse aggressive non-Hodgkin's lymphomas (NHL) included in the LNH87 protocol could be assessed for both morphology and immunophenotyping. Among them, 288 (15%) had PTCL and 1,595 (85%) had BCL. According to the Kiel classification, most PTCL were classified as angioimmunoblastic (AIL; 23%), pleomorphic medium and large T-cell (PML; 49%), or anaplastic large cell (ALCL; 20%) lymphomas. Comparing PTCL with BCL patients, the former had more disseminated disease (78% v 58%), B symptoms (57% v 40%), bone marrow involvement (31% v 17%), skin involvement (21% v 4%), and increased beta2-microglobulin (50% v 34%), whereas BCL patients had more bulky disease (41% v 26%). According to the International Prognostic Index (IPI), PTCL and BCL scores were, respectively: 0 factors, 13% and 15%; 1 factor, 17% and 22%; 2 factors, 24% and 25%; >/=3 factors, 45% and 37% (P = .09). For BCL and PTCL, respectively, complete remission rates were 63% and 54% (P = .004); the 5-year overall survival (OS) rates were 53% and 41% (P = .0004) and event-free survival (EFS) rates were 42% and 33% (P < . 0001). Comparison of the different histological subtypes of lymphoma showed that the 5-year OS rate for T-ALCL (64%) was superior to those of other PTCL (35%) as well as diffuse large B-cell (53%) NHL. When multivariate analysis was applied using the IPI score as one factor, nonanaplastic PTCL remained an independent parameter (P = . 0004). Although the poor prognosis of non-ALCL PTCL could be due in part to the presence of adverse prognostic factors at diagnosis, this study shows that the T-cell phenotype is an independent significant factor, which should be incorporated into the definition of prognostic groups.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Tumour 'bulk' has historically been considered an important prognostic marker and clinical tool to guide treatment in patients with lymphoma. However, its use and definitions in trial designs varies significantly and it is unclear how this has influenced the relevance of bulk in contemporary practice. This comprehensive literature review evaluated the definitions, applications and prognostic impact of bulk in phase 3 randomised trials in four major lymphoma subtypes.
View Article and Find Full Text PDFPeripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) is a rare mature T-cell non-Hodgkin lymphoma (NHL) seen in both children and adults. While it is the most common non-anaplastic mature T-cell lymphoma of childhood, it is quite rare and therefore, the standard of care remains largely undefined. It is a disease characterized by clinical and pathological heterogeneity and is generally associated with an aggressive clinical course and poor prognosis in adults.
View Article and Find Full Text PDFClinical imaging and pathology analysis of Epstein-Barr virus-associated monomorphic lymphoproliferative disease after liver transplantation in children-a retrospective case series.
Transl Pediatr
December 2024
Department of Hematology, Chongqing Medical University Affiliated Children's Hospital, Chongqing, China.
Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication that can arise following solid organ transplantation or hematopoietic stem cell transplantation. It encompasses a spectrum of lymphoproliferative lesions, ranging from benign reactive hyperplasia to malignant tumors, and is among the most severe complications following liver transplantation in children. It is essential for clinicians to gain a comprehensive understanding of the prevention, clinical manifestations, early diagnosis, and treatment strategies for PTLD in order to reduce mortality rates.
View Article and Find Full Text PDFBackground: Angioimmunoblastic T-cell lymphoma (AITL) is a distinct subtype of peripheral T-cell lymphoma (PTCL) and accounts for 2% of all non-Hodgkin lymphomas. Its typical characteristics include an aggressive course, progressive lymphadenopathy, hepatosplenomegaly, systemic symptoms, anemia, hypergammaglobulinemia, and generally poor prognosis.
Methods: We describe a rare case in which the left inguinal lymph node was completely excised and biopsied one year ago.
When the mask slips: A peripheral T-cell lymphoma disguised as lupus with myelofibrosis in a patient with May-Hegglin syndrome.
Leuk Res Rep
December 2024
Internal Medicine Department, Institut Jules Bordet - Hôpital Universitaire de Bruxelles (H.U.B.), Université Libre de Bruxelles (ULB), Brussels, Belgium.
We describe the case of a female patient with May-Hegglin syndrome who developed peripheral T-cell lymphoma not otherwise specified. The patient presents with systemic lupus erythematous phenotype and myelofibrosis secondary to T-cell lymphoma. Peripheral T-cell lymphoma not otherwise specified, represents 25 % of all peripheral T-cell lymphoma.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!