Odstrcil-Bobillo M Silvina, Kohan Dana, Heller Paula G, Otero Victoria, Russo María Paula, Basquiera Ana L
Departamento de Medicina Interna, Hospital Italiano de Buenos Aires, Hospital Italiano de Buenos Aires, Argentina. E-mail:
Servicio de Hematología, Hospital Italiano de Buenos Aires, Argentina
Medicina (B Aires). 2020;80(1):81-83.
Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.
威斯科特-奥尔德里奇综合征是一种罕见的X染色体连锁原发性免疫缺陷综合征,与感染、自身免疫性疾病和肿瘤的发病率增加有关。我们报告一例41岁男性,诊断为威斯科特-奥尔德里奇综合征,以回肠炎作为淋巴增殖综合征的一种表现形式。鉴于存在大量鉴别诊断(炎症性肠病、感染、肿瘤和淋巴增殖性疾病),该患者的回肠炎是一项临床挑战,因此通常需要进行解剖病理学诊断以及关于后续特定治疗的特殊考量。