Shenoy Shabari Mangalore, Di Vitantonio Thomas, Plitt Anna, Perez-Johnston Rocio, Gutierrez Jillian, Knorr David A, Stein Eytan M, Liu Jennifer E, Feldman Stephanie
Department of Medicine, Mount Sinai Morningside and West Hospital, Icahn School of Medicine at Mount Sinai, New York, USA.
Department of Medicine, New York Presbyterian/ Weill Cornell Medical Center, New York, USA.
Cardiooncology. 2021 Nov 23;7(1):39. doi: 10.1186/s40959-021-00124-9.
All trans retinoic acid (ATRA) has revolutionized the treatment and outcomes of patients with Acute Promyelocytic Leukemia (APL). Induction therapy with ATRA is associated with the rare but potentially fatal complication of differentiation syndrome. While the presentation of this syndrome is varied, myopericarditis as a manifestation of differentiation syndrome is often fatal and rarely reported in literature. We present a case of myopericarditis as the sole manifestation of differentiation syndrome in a patient on induction therapy with ATRA and arsenic trioxide for APL.
A 62 year old woman with remote history of breast and uterine cancer presented to the hospital for expedited work up of easy bruising and expanding hematomas. She was diagnosed with APL with peripheral blood and bone marrow cytogenetics revealing t (15;17) translocation and initiated on induction therapy with ATRA and ATO as well as steroids for differentiation syndrome prophylaxis. Eighteen days into induction therapy, patient developed pleuritic chest pain, elevated cardiac biomarkers, ECG changes suggestive of pericarditis. Cardiac magnetic resonance imaging showed patchy multifocal sub-epicardial late gadolinium enhancement and elevated T2 signal consistent with acute myopericarditis. Given the timing of symptom onset and lack of other identifiable cause, patient was diagnosed with differentiation syndrome- induced myopericarditis and promptly initiated on high dose steroids with rapid improvement in symptoms, ECG, and cardiac biomarkers. Patient successfully resumed dose-reduced ATRA and arsenic trioxide without complication.
Myopericarditis can be the sole manifestation of differentiation syndrome and the presentation may be atypical owing to the use of prophylactic steroids as illustrated in our patient's case. A high index of suspicion for differentiation syndrome, multimodality imaging, and prompt input from multidisciplinary providers is crucial for making the timely diagnosis and initiating life-saving treatment.
全反式维甲酸（ATRA）彻底改变了急性早幼粒细胞白血病（APL）患者的治疗方法及预后。ATRA诱导治疗与罕见但可能致命的分化综合征并发症相关。虽然该综合征的表现多样，但作为分化综合征表现形式的心肌心包炎往往是致命的，且在文献中鲜有报道。我们报告一例在接受ATRA和三氧化二砷诱导治疗的APL患者中，心肌心包炎作为分化综合征的唯一表现。
一名62岁有乳腺癌和子宫癌病史的女性因易出现瘀伤和血肿扩大而到医院进行快速检查。她被诊断为APL，外周血和骨髓细胞遗传学检查显示t（15；17）易位，并开始接受ATRA、ATO诱导治疗以及使用类固醇预防分化综合征。诱导治疗第18天，患者出现胸膜炎性胸痛、心脏生物标志物升高、心电图改变提示心包炎。心脏磁共振成像显示斑片状多灶性心外膜下晚期钆增强及T2信号升高，符合急性心肌心包炎表现。鉴于症状出现的时间及缺乏其他可识别病因，患者被诊断为分化综合征所致心肌心包炎，并立即开始使用大剂量类固醇治疗，症状、心电图及心脏生物标志物迅速改善。患者成功恢复减量的ATRA和三氧化二砷治疗，未出现并发症。
心肌心包炎可能是分化综合征的唯一表现，如我们患者的病例所示，由于使用了预防性类固醇，其表现可能不典型。对分化综合征保持高度怀疑、进行多模态成像检查以及多学科医疗团队及时介入，对于及时诊断和启动挽救生命的治疗至关重要。
