Conserva Maria Rosa, Redavid Immacolata, Anelli Luisa, Zagaria Antonella, Specchia Giorgina, Albano Francesco
Hematology Section, Department of Emergency and Organ Transplantation (D.E.T.O.), University of Bari, Bari, Italy.
Front Mol Biosci. 2019 Oct 24;6:114. doi: 10.3389/fmolb.2019.00114. eCollection 2019.
Retinoic acid receptor γ (RARγ) belongs to the nuclear receptor superfamily and shares 90% homology with retinoic acid receptor α (RARα) and retinoic acid receptor β (RARβ). rearrangements are well-known to be involved in acute promyelocytic leukemia (APL), but rearrangements can also resemble this kind of leukemia. In this review we trace the role of RARγ, considering both its physiological and oncogenic contribution; from 2011 to date, nine cases of patients harboring fusions have been reported. These patients showed typical APL features, including the clinical presentation, coagulation abnormalities and morphological features of bone marrow (BM), but are not responsive to APL standard therapy. We stress the urgent need for a better comprehension of the critical role of dysregulation in the leukemogenesis process, since optimum therapy strategies have not yet been established.
维甲酸受体γ（RARγ）属于核受体超家族，与维甲酸受体α（RARα）和维甲酸受体β（RARβ）具有90%的同源性。已知重排参与急性早幼粒细胞白血病（APL），但重排也可能类似这种白血病。在本综述中，我们追溯RARγ的作用，考虑其生理和致癌贡献；从2011年至今，已报道9例携带融合的患者。这些患者表现出典型的APL特征，包括临床表现、凝血异常和骨髓（BM）形态学特征，但对APL标准治疗无反应。我们强调迫切需要更好地理解失调在白血病发生过程中的关键作用，因为尚未确立最佳治疗策略。
