Kupelian Chloe, Sathi Bindu, Singh Deepika
Department of Pediatrics, Valley Children's Healthcare, Madera, CA, USA.
Department of Pediatric Hematology and Oncology, Valley Children's Healthcare, Madera, CA, USA.
Case Rep Rheumatol. 2021 Feb 27;2021:6640006. doi: 10.1155/2021/6640006. eCollection 2021.
Kawasaki disease is an acute multisystem vasculitis characterized by involvement of medium-sized vessels that mostly affects children under the age of 5 years. The presentation is typically preceded by five or more days of fever with additional clinical findings including rash, peripheral edema, mucositis, conjunctival changes, and unilateral cervical lymphadenopathy. The most feared complication of Kawasaki disease is development of coronary artery aneurysms. Common laboratory abnormalities include normocytic anemia, thrombocytosis, leukocytosis, and elevated inflammatory markers. Immune-mediated cytopenias such as autoimmune hemolytic anemia and thrombocytopenia are rarely seen at presentation in Kawasaki disease. We describe a unique case of a child presenting with autoimmune hemolytic anemia, who sequentially developed immune thrombocytopenia concerning for Evans' syndrome and eventually diagnosed with Kawasaki Disease with coronary artery dilatation. Characteristic clinical findings including extremity edema, cracked lips, and rash developed later in the course. Our patient was treated with IVIG and steroids with significant clinical improvement and complete resolution of cytopenias and coronary aneurysms on long term follow up. Timely administration of IVIG prevents and minimizes the risk of long term cardiac consequences. Hence a high index of suspicion should be maintained for this relatively common pediatric illness, even in absence of more commonly seen laboratory findings.
川崎病是一种急性多系统血管炎，其特征是中等大小血管受累，主要影响5岁以下儿童。典型表现通常是发热持续五天或更长时间，并伴有其他临床表现，包括皮疹、外周水肿、黏膜炎症、结膜改变和单侧颈部淋巴结病。川崎病最可怕的并发症是冠状动脉瘤的形成。常见的实验室异常包括正细胞性贫血、血小板增多、白细胞增多和炎症标志物升高。在川崎病发病时，很少见到免疫介导的血细胞减少症，如自身免疫性溶血性贫血和血小板减少症。我们描述了一例独特的病例，一名儿童最初表现为自身免疫性溶血性贫血，随后相继出现疑似伊文氏综合征的免疫性血小板减少症，最终被诊断为川崎病并伴有冠状动脉扩张。特征性的临床表现，包括肢体水肿、嘴唇干裂和皮疹，在病程后期出现。我们的患者接受了静脉注射免疫球蛋白（IVIG）和类固醇治疗，临床症状显著改善，血细胞减少症和冠状动脉瘤在长期随访中完全消退。及时给予IVIG可预防并降低长期心脏并发症的风险。因此，即使没有更常见的实验室检查结果，对于这种相对常见的儿科疾病也应保持高度怀疑。
