AUTHOR=Jiang Mingyan , Li Jinrong , Wu Jianrong , Zhu Yiping , Gao Ju 

TITLE=Case report: A rare case of TBL1XR1-RARB positive acute promyelocytic leukemia in child and review of the literature

JOURNAL=Frontiers in Oncology

VOLUME=Volume 12 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2022.1028089

DOI=10.3389/fonc.2022.1028089

ISSN=2234-943X

ABSTRACT=<p>Some forms of acute myelogenous leukemia (AML) share typical morphological and immunophenotypic features of acute promyelocytic leukemia (APL) but are negative for promyelocytic leukemia-retinoic acid receptor alpha (<italic>PML-RARA</italic>) fusion. These forms of AML are known as variant APL. Some variants of APL present with retinoic acid receptor beta (<italic>RARB</italic>) fused or rearranged with partner genes. <italic>RARB</italic>-positive APL is very rare, resistant to all-trans retinoic acid (ATRA), and associated with poor prognosis. Here, we reported one case with <italic>TBL1XR1-RARB</italic> positive APL, featured by early onset and no apparent bleeding tendency or coagulation dysfunction. This patient was resistant to ATRA and arsenic trioxide (ATO), but was good responsive to conventional chemotherapy for AML. The case report was followed by a literature review.</p>