Lymphoma of mucosa-associated lymphoid tissue in common variable immunodeficiency
Lymphoma of mucosa-associated lymphoid tissue in common variable immunodeficiency
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DOI：
10.1080/10428190500285285
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发表时间：
2006-02-01
影响因子：
2.6
通讯作者：
Webster, D
中科院分区：
文献类型：
--
作者：
Aghamohammadi, A;Parvaneh, N;Webster, D
常见变异性免疫缺陷（CVID）是最常见的症状性原发性免疫缺陷，其特征是所有主要免疫球蛋白类水平降低和复发性c感染。在不同的研究中发现，CVID患者发生非霍奇金淋巴瘤（NHL）的风险增加。粘膜相关淋巴组织（MALT）淋巴瘤是最近发现的一种由边缘区相关细胞组成的低级别b细胞NHL亚群。MALT淋巴瘤作为慢性炎症或自身免疫刺激的结果出现在淋巴组织中。本研究简要回顾了先前发表的病例，并报告了一名患有慢性腹泻和复发性肺感染的CVID患者。尽管静脉注射免疫球蛋白治疗，慢性咳嗽和喘息仍有进展。开放式肺活检显示MALT淋巴瘤。尽管肺部低级别b细胞淋巴瘤是一种罕见的并发症，但对于慢性肺部症状对常规治疗无反应的CVID患者，必须牢记这一诊断。
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.