Sabillón-Mendoza Alejandra Maricela, Imbett-Yepez Sharon María, Ignorosa-Arellano Karen, Cadena-León Jose Francisco, Zárate-Mondragón Flora, Cervantes-Bustamante Roberto, Ramírez-Mayans Jaime
Departamento de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Coyoacán, México.
Andes Pediatr. 2022 Jun;93(3):416-422. doi: 10.32641/andespediatr.v93i3.3676.
Chronic active Epstein Barr virus infection (CAEBV) is a rare condition, where the body is unable to counteract Epstein Barr viral replication (EBV), leading the patient to a chronic state with variable symptoms. Early recognition of infrequent or atypical clinical manifestations is relevant due to the particularities of their management and prognosis.
to describe a case of CAEBV manifes ted with colitis and hepatitis, summarizing the clinical-pathological and endoscopic characteristics and their evolution.
A 6-year-old girl, previously healthy, presented recurrent episodes of jaundice, hepatosplenomegaly, and fever. EBV hepatitis was diagnosed with a blood viral load of 328,000 copies / mL. Her liver biopsy revealed Epstein-Barr virus-encoded small RNAs (EBER). She evolved with mucosanguineous diarrhea and weight loss; the colonoscopy showed loss of the haustral pattern, multiple aphthous ulcers covered with fibrin, and 7 million copies of EBV / gram of tissue were found in the colon. T-cell lineage infection was identified, therefore Rituximab was started, with a decrease in viral load, complete resolution of diarrhea, and improvement in liver function tests. The definitive treatment was bone marrow transplantation.
CAEBV is a serious disor der, little documented, and should be considered in the face of a prolonged or intermittent course of hepatitis, accompanied by general and gastrointestinal manifestations such as chronic diarrhea, hematochezia, and weight loss, since its outcome without treatment can be fatal.
慢性活动性EB病毒感染(CAEBV)是一种罕见病症,机体无法抵抗EB病毒复制(EBV),导致患者处于具有多种症状的慢性状态。鉴于其治疗和预后的特殊性,早期识别罕见或非典型临床表现具有重要意义。
描述一例表现为结肠炎和肝炎的CAEBV病例,总结临床病理及内镜特征及其演变过程。
一名6岁以前健康的女孩出现黄疸、肝脾肿大和发热反复发作。通过血液病毒载量328,000拷贝/毫升诊断为EBV肝炎。她的肝脏活检显示EB病毒编码的小RNA(EBER)。她出现了黏液血性腹泻和体重减轻;结肠镜检查显示结肠袋消失,多个覆盖有纤维蛋白的阿弗他溃疡,并且在结肠中发现每克组织有700万拷贝的EBV。确定为T细胞谱系感染,因此开始使用利妥昔单抗治疗,病毒载量下降,腹泻完全缓解,肝功能检查改善。最终治疗是进行骨髓移植。
CAEBV是一种严重疾病,文献记载较少,当出现肝炎的长期或间歇性病程,并伴有慢性腹泻、便血和体重减轻等全身及胃肠道表现时应考虑该病,因为未经治疗其后果可能是致命的。