Division of Pediatric Oncology, Catholic University of Rome, Rome, Italy.
Pediatr Blood Cancer. 2012 Aug;59(2):318-9. doi: 10.1002/pbc.23393. Epub 2011 Nov 2.
Wiskott-Aldrich syndrome (WAS) is characterized by primary immunodeficiency, thrombocytopenia and eczema. Patients with WAS have an increased risk to develop tumors. Non-Hodgkin lymphoma (NHL) represents the most common malignancy occurring in WAS-affected patients, diffuse-large-B-cell lymphoma is the most frequently encountered variant. We describe a case of a patient with WAS and NHL in the pharynx, an atypical tumor site presentation. The patient was successfully treated with a reduced dose chemotherapy regimen plus anti-CD20 monoclonal antibody. He is in complete remission 3 years from the start of treatment.
威斯科特-奥尔德里奇综合征(WAS)的特征为原发性免疫缺陷、血小板减少和湿疹。WAS 患者发生肿瘤的风险增加。非霍奇金淋巴瘤(NHL)是非 WAS 相关患者最常见的恶性肿瘤,弥漫性大 B 细胞淋巴瘤是最常遇到的变体。我们描述了一例 WAS 合并 NHL 发生于咽部的病例,这是一种非典型的肿瘤部位表现。该患者接受了降低剂量的化疗方案加抗 CD20 单克隆抗体治疗,取得了成功。自治疗开始 3 年后,他处于完全缓解状态。