Perifollicular Variant of Peripheral T-Cell Lymphoma, UNS: A Distinct Lymphoma Type with Cytogenetic andcytogenetic and Clinical Findings Different from Those of Angio-Immunoblastic Lymphoma
Notice bibliographique
Résumé
Abstract Perifollicular variant of peripheral T-cell lymphoma, UNS: a distinct lymphoma type with cytogenetic and clinical findings different from those of angio-immunoblastic lymphoma. A. Bassarova, F. Micci, G. Trzen, H. Holte, J. Delabie Peripheral T-cell lymphoma, unspecified (PTCL-U), represents approximately 30% of all peripheral T-cell lymphomas (PTCL) in adults, excluding primary cutaneous lymphomas. PTCL-U represents approximately 12–15% of lymphomas in the Western population. The disease has a five-year overall survival of about 30%. PTCL-U is currently an amalgam of T-cell lymphomas with a mature immunophenotype that cannot be classified into other well-characterized types of PTCL. However, within PTCL-U several variants are tentatively recognized, among which the follicular or perifollicular variant. This entity is histologically characterized by its localization within the B-cell area of the lymph node and by its T follicular helper cell immunophenotype. In view of the latter, it has been argued that the follicular variant of PTCL-U is an early stage of angio-immunoblastic T-cell lymphoma, a well-characterized type of PTCL of T follicular helper cell origin. To better study perifollicar T-cell lymphoma as compared to AITL, we retrieved 47 cases of PTCL with these diagnoses from the archive of the Department of Pathology at Oslo University Hospital collected in a time period from 1999 to 2013 and reviewed the morphological, immunophenotypic, cytogenetic and clinical findings. Immunophenotypic analysis was completed when necessary. Diagnoses were made according to the W.H.O. criteria that included those published by our group previously. Cytogenetic data were available for 39 cases. Nineteen cases of perifollicular TCL and 23 cases of AITL were retrieved and 4 cases with features between perifollicular TCL and AITL were identified. By cytogenetic analysis, perifollicular TCL showed a complex karyotype with structural and numerical abnormalities. The most frequently observed aberrations were loss of 4p16 and 6q22 as well as gains of 1q21 and 3q11q12 (Fig. 1). AITL was characterized by predominantly numerical chromosomal aberrations and less structural abnormalities. The most frequent detected abnormalities were gains of chromosome 3, 5, 7, 9 and 19 (Fig. 2). The patients with perifollicular TCL had normal to high lymphocytic counts (p<0.05), platelet counts greater than 145 (p<0.001), normal LDH levels, albumin levels above the median (p<0.05) normal or slightly elevated sedimentation ratios (p<0.05), normal or above median hemoglobin levels (p<0.001) and infrequent bone marrow involvement at presentation. The majority of patients had international prognostic index (IPI) scores of 0, 1 or 2 (p<0.05) and a W.H.O. performance status between 0 and 2. Bulky disease as well as extranodal involvement at presentation was rare in this group of patients. By contrast patients with AITL presented with B-symptoms, high sedimentation ratios (p<0.05), lymphopenia (p<0.05), thrombocytopenia (p<0.001), elevated LDH and low hemoglobin levels (p<0.001) and hypoalbuminemia (p<0.05). The patients had IPI scores between 3 and 5 at presentation (p<0.05). All patients with AITL had bulky disease and 40 % had multiple extranodal localizations at presentation. Importantly, patients with perifollicular TCL showed better response to therapy and had significantly longer overall survival when compared with AILT (Fig. 3). In conclusion, the cytogenetic findings showing a complex karyotype with predominantly structural alterations, the less severe symptoms and signs at presentation and the longer overall survival are features that distinguish perifollicular TCL from AITL. Together these features do not favor the hypothesis that perifollicular TCL is an early stage of AITL but indicate that perifollicular TCL is a distinct type of PTCL. The identification of four cases with histological features between those of perifollicular TCL and AITL in our series may indicate that better methods are needed to reliably separate these entities. Therefore, detailed genetic characterization of perifollicular TCL in comparison with AITL is currently being undertaken. Fig. 1 Schematic representation of the cytogenetic data of PTCL-perifollicular Fig. 1. Schematic representation of the cytogenetic data of PTCL-perifollicular Fig. 2 Schematic representation of the cytogenetic data of AITL Fig. 2. Schematic representation of the cytogenetic data of AITL Fig. 3 The overall survival of PTCL-perifollicular vs. AITL Fig. 3. The overall survival of PTCL-perifollicular vs. AITL Disclosures No relevant conflicts of interest to declare.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,001 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,001 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».