Outcomes of Mixed Phenotype Leukemia, Not Otherwise Specified (NOS), in Adults: A Single Centre Retrospective Review from 2000 to 2014
Notice bibliographique
Résumé
Abstract Introduction: Acute leukemias of ambiguous lineage (AUL), biphenotypic leukemias (BAL) and mixed phenotypic leukemias (MPAL) are a heterogeneous group of rare, poorly characterized leukemias with unfavorable outcomes. AUL, BAL and MPALs are believed to constitute 2-5% of all leukemias. The optimal treatment regime for this group of leukemias is currently unknown. The primary objective of this study is to determine the complete remission rate (CR) and overall survival (OS) among adult patients with mixed phenotypic leukemia, not otherwise specified (NOS), as defined by the WHO 2008 classification criteria. Methods: Patients with mixed phenotype acute leukemia (MPAL) older than 17 years of age who were treated at Princess Margaret Cancer Centre from January 1, 2000 to June 30, 2014 were identified through the Leukemia Database at Princess Margaret Cancer Registry. Pathology review of the original diagnostic bone marrow was completed to ensure patients met the WHO 2008 criteria for MPAL, not otherwise specified (NOS). Patients with MLL+, BCR-ABL+, PDGRFA+ were excluded from analysis. Results: Fourteen patients were identified and confirmed by pathology review. Eleven (79%) were male, 3 (29%) were female. The median age was 57 years (range: 18-84). Eight (57%) had extramedullary involvement at presentation. Six (42%) had poor risk cytogenetics, whereas 8 (57%) had intermediate risk cytogenetics at presentation as per the MRC criteria. Two patients (14%) had p53 mutations/loss of chromosome 17. Four patients (42%) had monosomy 7 karyotype. Six cases (43%) were myeloid/B, seven cases (50%) were myeloid/T and one (7%) was myeloid/T/B type. The median WBC at presentation was 10.8 x 109/L (2.3 x 109/L-106 x 109/L). Five patients were treated upfront with daunorubicin and cytarabine (3&7) with 1 of these patients achieved a CR with this upfront regime. Five patients were treated upfront with the Dana Farber Consortium Protocol (DFCI) and 4 of these patients achieved a CR with this upfront regime. Two patients were treated upfront with HyperCVAD, and 1 achieved a CR. Two patients were treated palliatively. One received azacytidine as palliative treatment and achieved a CR with a duration of 5 months. Ten (71%) of these patients achieved a CR. Three of the primary nonresponders achieved a CR with an alternative induction regime. The median CR1 duration was 5.5 mos (range, 2-40 mos). Overall median survival was 13 mos (95% CI, 8 mos-27 mos). The OS at 1 year and 2 years was 56% (95% CI, 29%- 82%) and 32% (95% CI, 6%-57%), respectively. Four patients (29%) underwent an allogeneic hematopoietic stem cell transplant. At last follow up, 13 of the 14 patients have died, 11 from relapsed leukemia and 2 from sepsis post HCT. One patient died of relapsed leukemia post HCT. Conclusions: This retrospective review confirms the high risk nature and poor prognosis of MPAL NOS. Our limited cohort showed a male predominance, a high rate of extramedullary involvement at presentation, and a higher rate of CR with DFCI as the initial remission induction regime. HCT or other forms of maintenance therapy should be considered in CR1 in eligible patients due to the high- risk nature of MPAL NOS. Disclosures Gupta: Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees; Incyte: Honoraria, Research Funding.
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 | 0,001 | 0,003 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,002 | 0,002 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,001 | 0,001 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,001 | 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 ».