Aghamohammadi Asghar, Parvaneh Nima, Tirgari Farrokh, Mahjoob Fatemeh, Movahedi Masoud, Gharagozlou Mohammad, Mansouri Mahboubeh, Kouhi Ali, Rezaei Nima, Webster David
Department of Clinical Pediatric Immunology, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Leuk Lymphoma. 2006 Feb;47(2):343-6. doi: 10.1080/10428190500285285.
Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency characterized by reduced levels of all major immunoglobuline classes and recurrent c infections. The risk of non-Hodgkin's lymphoma (NHL) among patients with CVID was found to be increased in different studies. Mucosa-associated lymphoid tissue (MALT) lymphomas are a recently recognized sub-set of low-grade B-cell NHL composed of marginal zone-related cells. MALT lymphomas appear in the lymphoid tissues as a result of chronic inflammatory or autoimmune stimulation. This study briefly reviews previously published cases and reports a patient suffering from CVID with a history of chronic diarrhea and recurrent sinopulmonary infections. Despite treatment with intravenous immunoglobulin, chronic cough and wheezing progressed. Open lung biopsy showed a MALT lymphoma. Although a rare complication, pulmonary low grade B-cell lymphoma is a diagnosis that must be kept in mind in CVID patients with chronic pulmonary symptoms unresponsive to conventional therapies.
普通可变免疫缺陷（CVID）是最常见的有症状的原发性免疫缺陷，其特征是所有主要免疫球蛋白类别的水平降低以及反复感染。在不同研究中发现，CVID患者中非霍奇金淋巴瘤（NHL）的风险增加。黏膜相关淋巴组织（MALT）淋巴瘤是最近才被认识到的由边缘区相关细胞组成的低度B细胞NHL的一个子集。MALT淋巴瘤由于慢性炎症或自身免疫刺激而出现在淋巴组织中。本研究简要回顾了先前发表的病例，并报告了一名患有CVID的患者，有慢性腹泻和反复鼻窦肺部感染史。尽管接受了静脉注射免疫球蛋白治疗，但慢性咳嗽和喘息仍在进展。开放性肺活检显示为MALT淋巴瘤。尽管是一种罕见的并发症，但对于有慢性肺部症状且对传统疗法无反应的CVID患者，肺部低度B细胞淋巴瘤是一个必须考虑的诊断。
