Cancers, Vol. 12, Pages 1313: PLZF-RAR α, NPM1-RARα, and Other Acute Promyelocytic Leukemia Variants: The PETHEMA Registry Experience and Systematic Literature Review
Cancers, Vol. 12, Pages 1313: PLZF-RARα, NPM1-RARα, and Other Acute Promyelocytic Leukemia Variants: The PETHEMA Registry Experience and Systematic Literature Review
Cancers doi: 10.3390/cancers12051313
Authors:
Marta Sobas
Maria Carme Talarn-Forcadell
David Martínez-Cuadrón
Lourdes Escoda
María J. García-Pérez
Jose Mariz
María J. Mela-Osorio
Isolda Fernández
Juan M. Alonso-Domínguez
Javier Cornago-Navascués
Gabriela Rodríguez-Macias
María E. Amutio
Carlos Rodríguez-Medina
Jordi Esteve
Agnieszka Sokół
Thais Murciano-Carrillo
María J. Calasanz
Manuel Barrios
Eva Barragán
Miguel A. Sanz
Pau Montesinos
It has been suggested that 1–2% of acute promyelocytic leukemia (APL) patients present variant rearrangements of retinoic acid receptor alpha (RARα) fusion gene, with the promyelocytic leukaemia zinc finger (PLZF)/RARα being the most frequent. Resistance to all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO) has been suggested in PLZF/RARα and other variant APLs. Herein, we analyze the incidence, characteristics, and outcomes of variant APLs reported to the multinational PETHEMA (Programa para el Tratamiento de Hemopatias Malignas) registry, and we perform a systematic review in order to shed light on strategies to improve management of these extremely rare diseases. Of 2895 patients with genetically confirmed APL in the PETHEMA registry, 11 had variant A...
Source: Cancers - Category: Cancer & Oncology Authors: Marta Sobas Maria Carme Talarn-Forcadell David Mart ínez-Cuadrón Lourdes Escoda Mar ía J. García-Pérez Jose Mariz Mar ía J. Mela-Osorio Isolda Fern ández Juan M. Alonso-Dom ínguez Javier Cornago-Navascu és Gabriela Rodr íguez-Macias Mar ía E. A Tags: Review Source Type: research
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