Retrospective Study of Intravascular Lymphoma Cases Diagnosed in Quebec
Notice bibliographique
Résumé
Abstract Intravascular lymphoma (IVL) is characterized by exclusive or predominant presence of lymphoma cells in the lumina of small vessels. Even though T-cell phenotype has been reported, IVL has recently been classified by the World Health Organization (WHO) Classification as a subtype of diffuse large B-cell lymphoma (DLBCL). IVL is considered an aggressive, disseminated and usually fatal malignancy that affects older individuals. Its initial clinical presentation varies according to geography. "Classical" IVL, mainly reported in Europe, is characterized by cutaneous and/or neurological involvement. "Asian variant" IVL, mainly reported in Asia, is characterized by hemophagocytosis, bone marrow (BM) involvement, fever, hepatosplenomegaly and/or thrombocytopenia. This heterogeneous clinical presentation of IVL often causes a delay in diagnosis and treatment instauration. IVL has been mainly studied through European and Asian cases. However, the majority of these case series and cumulative reviews have been based on a restricted number of patients, limiting the clinicopathological understanding and treatment options of this disease. Given the lack of previous large North American studies, we decided to analyze a case series in our country (Canada, Québec) in order to compare our results with the European and Asian series. We report the largest North American study to date on IVL. We reviewed all IVL cases in all Quebec hospitals between 1990 and 2016 (n= 29). The aim of our study was to describe the clinical presentations, investigations, therapeutic management and clinical outcomes of IVL patients in our population and compare the disease phenotype to European and Asian cases. In our cohort, 100% of patients had stage IV IVL at diagnosis, with a median age of 66.7 years (range 47.2-90.8). Clinical presentation was characterized by constitutional symptoms (100%), poor ECOG-PS (100% ≥ 2), cytopenias (93% anemia) and elevated lactate dehydrogenase (97%) and C-reactive protein (96%). Our cohort presented with mainly cutaneous and neurological symptoms. However, neurological involvement (75.9%) was predominant and no "cutaneous variant" was observed; this differs from European literature, where "classical" IVL is reported with mainly cutaneous involvement. Two of our Caucasian patients presented "Asian variant" IVL; this observation is not unusual, as cases of "classical" IVL have been reported in Asians and "Asian variant" IVL has been reported in Europeans. All patients were classified according to their immunophenotypic features in 3 different subgroups (CD5+ or CD5-CD10+, CD5-CD10-, CD5+CD10-). According to the literature, no significant difference between these subgroups was found. Finally, 62% of our cohort received anthracycline-based chemotherapy, and within this group, 53% and 20% of patients achieved a complete and partial response, respectively. Overall survival at 3 years was 42.7% and 47.4% for the entire cohort and for the cases with in vivo diagnosis, respectively. Event-free survival at 3 years was 64.2%. Median follow-up of our cohort was 328 days (CI 181-474). Unlike European studies on "classical" IVL, our study showed that the Canadian presentation of this subtype of IVL is more frequently observed with neurological rather than cutaneous involvement. Based on the fact that two of our Caucasian patients presented "Asian variant" IVL, we suggest that the IVL nomenclature should use "classical" IVL and "IVL associated with haemophagocytic syndrome", instead of "European" IVL and "Asian variant" IVL. Disclosures Fleury: Novartis: Consultancy, Speakers Bureau; Amgen: Consultancy, Speakers Bureau; Gilead: Consultancy, Speakers Bureau; Seattle Genetics: Consultancy, Speakers Bureau; Roche: Consultancy, Speakers Bureau; Janssen: Consultancy, Speakers Bureau; Lundbeck: Consultancy, Speakers Bureau.
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 | 0,003 | 0,003 |
| Études des sciences et des technologies | 0,002 | 0,001 |
| Communication savante | 0,001 | 0,000 |
| Science ouverte | 0,001 | 0,001 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,003 | 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 ».