ABCL-262 Diffuse Large B Cell Lymphoma in a Child With Epidermodysplasia Verruciformis, Case Report

Context Epidermodysplasia verruciformis (EV) is a rare skin disorder inherited as an autosomal recessive disorder. It is usually associated with genetic alteration and immunodeficiency. Patients are susceptible to human papillomavirus (HPV) infection. There are very few reports about its association with hematological malignancies, and those were reported only in adults. Patient We reviewed the records of an eleven-year-old child who presented to the National Cancer Institute, Cairo University, in August 2021. Results The patient came to the outpatient clinic with a history of being treated for skin disease since he was 5 years old. He had lesions affecting his face, trunk, and both upper limbs in the form of brown and red papules and plaques. He sought medical advice, and a skin biopsy revealed he had EV. He was treated with topicals, oral antihistaminics, and antibiotics. He presented with complaints of fever, dry cough, and weight loss of 4 months, for which he received nonspecific medications without improvement. Chest X-ray revealed right-sided pleural effusion with lung collapse. He was admitted and did CT, which revealed right ill-defined hypodense lesions involving the whole lower lobe and, to a lesser extent, upper and middle lobe with mild pleural effusion associated with multiple mediastinal lymph nodes and left lung pulmonary nodules. A biopsy was done, and pathology revealed diffuse large B cell lymphoma. Bone marrow aspirate and biopsy were free. Other investigations were normal. The patient decided to start chemotherapy on LMB protocol. He received pre-phase chemotherapy (COP) with more than a 20% reduction in the previously described lesions. He then received 2 cycles of COPADM3 and one CYM with almost complete resolution of the lesions. He received the last Cycle CYM and is now following up. Conclusion EV may predispose to lymphomagenesis due to its association with immunosuppression and with HPV infection that has lymphoma oncogenesis.

背景 疣状表皮发育不良（EV）是一种罕见的常染色体隐性遗传性皮肤病。它通常与基因改变和免疫缺陷有关。患者易感染人乳头瘤病毒（HPV）。关于其与血液系统恶性肿瘤相关性的报道极少，且仅见于成人。 患者 我们回顾了一名11岁儿童的病历，该儿童于2021年8月就诊于开罗大学国家癌症研究所。 结果 患者因自5岁起就接受皮肤病治疗而来到门诊。他的面部、躯干和双上肢有病变，表现为棕色和红色丘疹及斑块。他寻求医疗建议，皮肤活检显示他患有EV。他接受了外用药物、口服抗组胺药和抗生素治疗。他因发热、干咳和体重减轻4个月前来就诊，为此他接受了非特异性药物治疗但无改善。胸部X线显示右侧胸腔积液伴肺不张。他入院并进行了CT检查，结果显示右侧边界不清的低密度病变累及整个下叶，在较小程度上累及上叶和中叶，伴有轻度胸腔积液以及多个纵隔淋巴结和左肺肺结节。进行了活检，病理显示弥漫性大B细胞淋巴瘤。骨髓穿刺和活检无异常。其他检查均正常。患者决定按照LMB方案开始化疗。他接受了前期化疗（COP），之前描述的病变减少了20%以上。然后他接受了2个周期的COPADM3和1个CYM治疗，病变几乎完全消退。他接受了最后一个CYM周期治疗，目前正在随访。 结论 由于EV与免疫抑制以及具有淋巴瘤致癌作用的HPV感染相关，它可能诱发淋巴瘤发生。