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: Tags: Review Source Type: research