■本研究旨在阐明最初表现为心脏压塞的小儿急性髓细胞性白血病(AML)的临床特征,并分享治疗经验。
■五名儿科患者最初被诊断为AML并伴有心脏髓样肉瘤(MS)。诊断是通过检查我们的医院记录并回顾1990年至2023年7月的相关文献来建立的,可通过MEDLINE/PubMed访问。我们全面评估了这些患者的临床特征和治疗方式。
■5名儿科患者出现急性症状,包括呼吸急促,萎靡不振,咳嗽,发烧,导致他们住院。体格检查显示烦躁,缺氧,呼吸急促,心动过速,和低血压。初始检测利用胸部X光或超声心动图,导致基于心包积液和/或骨髓检查的后续诊断。两名患者在最初诊断时接受了化疗,一种是阿糖胞苷和依托泊苷,另一种是阿糖胞苷和克拉屈滨。后处理,他们的骨髓得到了缓解,在2.5年的随访中,他们的心脏功能仍然良好。不幸的是,其余三名患者在诊断后两周内死亡,由于接受替代药物或没有接受化疗。
■这是第一个也是最大的小儿AML合并心脏MS的病例系列,最初表现为心脏填塞。它强调了与这种情况相关的罕见性和高死亡率。降低死亡率的关键因素包括确定临床表现,进行彻底的身体检查,及时进行超声心动图检查,早期和及时启动心包引流,避免心脏毒性化疗药物。
UNASSIGNED: This study aims to elucidate the clinical features observed in cases of pediatric acute myeloid leukemia (AML) initially presenting with cardiac tamponade and to share treatment experiences.
UNASSIGNED: Five pediatric patients were initially diagnosed with AML accompanied by cardiac myeloid sarcoma (MS). The diagnosis was established by examining our hospital records and reviewing pertinent literature from 1990 to July 2023, accessible through MEDLINE/PubMed. We comprehensively assessed the clinical characteristics and treatment modalities employed for these patients.
UNASSIGNED: Five pediatric patients presented with acute symptoms, including shortness of breath, malaise, cough, and fever, leading to their hospitalization. Physical examination revealed irritability, hypoxia, tachypnea, tachycardia, and hypotension. Initial detection utilized chest X-ray or echocardiogram, leading to subsequent diagnoses based on pericardial effusion and/or bone marrow examination. Two patients received chemotherapy at the time of initial diagnosis, one with cytarabine and etoposide, and the other with cytarabine and cladribine. Post-treatment, their bone marrow achieved remission, and over a 2.5-year follow-up, their cardiac function remained favorable. Unfortunately, the remaining three patients succumbed within two weeks after diagnosis, either due to receiving alternative drugs or without undergoing chemotherapy.
UNASSIGNED: This is the first and largest case series of pediatric AML patients with cardiac MS, manifesting initially with cardiac tamponade. It highlights the rarity and high mortality associated with this condition. The critical factors for reducing mortality include identifying clinical manifestations, conducting thorough physical examinations, performing echocardiography promptly, initiating early and timely pericardial drainage, and avoiding cardiotoxic chemotherapy medications.