MacDougall Kira, Chukkalore Divya, Rehan Maryam, Kashi Meena, Bershadskiy Alexander
Department of Internal Medicine, Zucker School of Medicine at Hofstra/Northwell at Staten Island University Hospital, New York, NY, USA.
Division of Hematology & Medical Oncology, Zucker School of Medicine at Hofstra/Northwell at Staten Island University Hospital, New York, NY, USA.
J Community Hosp Intern Med Perspect. 2021 Nov 15;11(6):832-838. doi: 10.1080/20009666.2021.1973657. eCollection 2021.
Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia characterized by a translocation of chromosomes 15 and 17, creating an alternation in the retinoic acid receptor-alpha (RAR-alpha) gene. This leads to excessive medullary production of promyelocytic blasts, which are frequently associated with the hemorrhagic complications seen in APL. In contrast, APL-associated thrombosis occurs much less frequently and is an underappreciated life-threatening manifestation of the disease. Most thrombotic events occur during induction chemotherapy with all-transretinoic acid and are rarely seen as the initial presentation on APL. Here we report an exceedingly rare case of a patient with recurrent venous and arterial thrombotic events, including deep vein thrombosis, bilateral segmental pulmonary embolism, an ischemic stroke, splenic infarcts, and renal infarcts, later found to have APL. We aim to discuss the most recent understanding of the pathogenesis of APL-associated thrombosis and to summarize the literature of this rare presentation of APL.
急性早幼粒细胞白血病(APL)是急性髓系白血病的一种独特亚型,其特征是15号和17号染色体易位,导致维甲酸受体α(RAR-α)基因发生改变。这会导致骨髓中早幼粒细胞大量增殖,这些细胞常与APL中出现的出血并发症相关。相比之下,APL相关的血栓形成发生率要低得多,是该疾病一种未得到充分认识的危及生命的表现。大多数血栓事件发生在使用全反式维甲酸进行诱导化疗期间,很少作为APL的初始表现出现。在此,我们报告一例极为罕见的病例,患者反复出现静脉和动脉血栓事件,包括深静脉血栓形成、双侧节段性肺栓塞、缺血性中风、脾梗死和肾梗死,后来被诊断为APL。我们旨在讨论对APL相关血栓形成发病机制的最新认识,并总结关于APL这种罕见表现的文献。