Naeem Mohtashim, Harrison Kathleen, Barton Kevin, Nand Sucha, Alkan Serhan
Department of Pathology, Loyola University Medical Center, Maywood, IL 60153, USA.
Eur J Haematol. 2006 Feb;76(2):164-6. doi: 10.1111/j.0902-4441.2005.00583.x.
Acute promyelocytic leukemia (PML) is characterized by a reciprocal translocation between chromosomes 15 and 17 resulting in a chimeric PML and retinoic acid receptor alpha (RARA) oncogene. The resultant fusion protein (PML/RARA) is thought to block differentiation of bone marrow cells arrested at the promyelocytic stage. In vitro and in vivo studies have shown that the large majority of APL cells undergo granulocytic maturation after ATRA therapy. We report a unique case of a PML/RARA positive APL patient exhibiting extensive monocytic differentiation after ATRA therapy as documented by morphology, flow cytometry, and FISH studies. We discuss potential dual capability for granulocytic/monocytic differentiation of PML/RARA positive APL cells and implications of monocytic differentiation in the management of APL patients treated with ATRA.
急性早幼粒细胞白血病(PML)的特征是15号和17号染色体之间发生相互易位,导致嵌合的早幼粒细胞白血病(PML)和维甲酸受体α(RARA)癌基因。由此产生的融合蛋白(PML/RARA)被认为会阻断停滞在早幼粒细胞阶段的骨髓细胞的分化。体外和体内研究表明,绝大多数急性早幼粒细胞白血病(APL)细胞在全反式维甲酸(ATRA)治疗后会经历粒细胞成熟。我们报告了一例独特的PML/RARA阳性急性早幼粒细胞白血病(APL)患者,经形态学、流式细胞术和荧光原位杂交(FISH)研究证实,该患者在全反式维甲酸(ATRA)治疗后表现出广泛的单核细胞分化。我们讨论了PML/RARA阳性急性早幼粒细胞白血病(APL)细胞粒细胞/单核细胞分化的潜在双重能力以及单核细胞分化在接受全反式维甲酸(ATRA)治疗的急性早幼粒细胞白血病(APL)患者管理中的意义。