CD27是一个辅助刺激受体，涉及先天和适应性免疫的成熟。CD27通过与CD70相互作用，在控制EBV感染中发挥作用。CD27缺陷导致EBV易感的免疫调节疾病。SARS-CoV-2可能会使原发性免疫缺陷患者面临不良结局的风险。用染色原位杂交（CISH）研究检测淋巴瘤组织中的EBV。患者的遗传学分析使用全外显子测序进行，检测到的变异并用PCR-Sanger测序得到确认。这里我们报道了一名20个月大的CD27缺陷男孩，他患有淋巴瘤和冠状动脉畸形，并感染了SARS-CoV-2。临床和实验室检查结果与不典型的川崎综合征或儿童多系统炎症综合征（MIS-C）不符。由于CD27缺陷是一种罕见的免疫缺陷，发布有关已确定患者的临床数据可以使我们更加了解与CD27缺陷相关的表型和临床表现谱。因此，我们的发现拓展了表现谱，除了EBV感染之外，还突出了这种可能与EBV感染、淋巴瘤或潜在疾病有关的不寻常心脏后遗症。

CD27 is a costimulatory receptor involved in the maturation of the innate and adaptive immunity. CD27, through interaction with CD70, plays a role in the control of Epstein-Barr virus (EBV) infection. CD27 deficiency leads to an immune dysregulation disease characterized by EBV susceptibility. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) might put patients with primary immunodeficiency at risk for adverse outcomes. Chromogenic in situ hybridization (CISH) study was performed to detect EBV in the lymphoma tissue. Genetic analysis of the patient was done with Whole Exome Sequencing and detected variant was confirmed with PCR-Sanger sequencing. Here we report a 20-month-old boy with CD27 deficiency who developed lymphoma and coronary artery ectasia and had been infected with SARS-CoV-2. Clinical and laboratory findings were incompatible with atypical Kawasaki syndrome or multisystem inflammatory syndrome in children (MIS-C). As CD27 deficiency is a rare immune defect, publishing clinical data about the identified patient(s) can shed light on our knowledge about the related phenotype and the spectrum of clinical manifestations associated with CD27 deficiency. Thus, our findings expanded the spectrum of manifestations beyond EBV infection, highlighting this unusual cardiac sequela that could be related to EBV infection, lymphoma, or an underlying disease.