Bronchus-associated Lymphoid Tissue in Kabuki Syndrome with Associated Hyper-IgM Syndrome/Common Variable Immunodeficiency.
Bronchus-associated Lymphoid Tissue in Kabuki Syndrome with Associated Hyper-IgM Syndrome/Common Variable Immunodeficiency.
复制标题
歌舞伎综合征与相关高 IgM 综合征/常见变异性免疫缺陷中的支气管相关淋巴组织。
DOI：
10.1164/rccm.201511-2305im
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发表时间：
2016
影响因子：
24.7
通讯作者：
Merlo,ChristianA
中科院分区：
文献类型：
--
作者：
Mock,JasonR;Kolb,ToddM;Illei,PeterB;Yang,StephenC;Lederman,HowardM;Merlo,ChristianA
图 1.(A) 胸部冠状位计算机断层扫描图像显示，一名 28 岁女性的肺底结节性浸润更加明显，该女性有 3 个月的呼吸困难和干咳病史。(B) 视频辅助胸外科活检的组织学切片显示结节性淋巴样增生，滤泡以小气道和机化性肺炎区域为中心。免疫染色显示，滤泡由 CD3 1 T 细胞组成，周围是 CD20 1 B 细胞的中心组（放大 320 倍）。插图代表较低放大倍数部分（3200 放大倍数）中淋巴滤泡的连续免疫染色。(C) 给予硫唑嘌呤和利妥昔单抗一个月后，冠状计算机断层扫描图像的结果显着改善。 H&E = 苏木精和伊红。 一名 28 岁女性，有明显的歌舞伎综合征病史并伴有高 IgM 综合征/常见变异型免疫缺陷，有 3 个月的呼吸困难、胸膜炎性疼痛和干咳病史。影像学显示结节性浸润向肺基底方向增加（图 1A）。进行支气管镜检查、灌洗和经支气管活检以评估感染性或炎症性病因；然而，结果并不明确，也没有发现传染性病因。因此，她接受了电视辅助胸外科活检，结果显示结节性淋巴组织增生，滤泡集中在小气道和机化性肺炎区域（图 1B）。免疫染色显示滤泡由 CD3 1 T 细胞和 CD20 1 B 细胞组成（图 1B），并且没有淋巴瘤或浆细胞肿瘤的形态学证据。结果与支气管相关淋巴组织（BALT）一致。使用高剂量类固醇后，症状或影像学结果并未得到缓解。一份关于利妥昔单抗和硫唑嘌呤对常见变异性免疫缺陷患者有效缓解的报告已发表，这些患者具有类似的肺部炎症过程，包括三级淋巴结构、肉芽肿和机化性肺炎 (1)。服用四剂利妥昔单抗（每周）并开始服用硫唑嘌呤后，她的症状和影像学结果有所改善（图 1C）。 BALT 是一种三级淋巴组织，由 Bienenstock 于 1973 年首次命名 (2)，并被描述为气道分叉处淋巴和结构细胞的滤泡样聚集 (3)。三级淋巴组织不同于初级（例如胸腺或骨髓）和二级（例如淋巴结或脾脏）淋巴组织 (3)。三级淋巴组织异位发育，主要在粘膜部位，可由自身免疫或慢性炎症引发（综述见参考文献 3）。 BALT 形成于
Figure 1.(A) Coronal chest computed tomography images that demonstrated nodular infiltrates more prominent toward the lung bases in a 28-year-old woman with a 3-month history of dyspnea and a nonproductive cough.(B) Histological sections from video-assisted thoracic surgery biopsy demonstrated nodular lymphoid hyperplasia with follicles centered on small airways and areas of organizing pneumonia. Immunostains demonstrated follicles composed of CD3 1 T cells surrounding a center grouping of CD20 1 B cells (320 magnification). Insets represent serial immunostains of a lymphoid follicle in the lower magnification section (3200 magnification).(C) One month after administration of azathioprine and rituximab, findings on coronal computed tomography images were dramatically improved. H&E= hematoxylin and eosin.A 28-year-old woman with a medical history significant for Kabuki syndrome with associated hyper-IgM syndrome/common variable immunodeficiency presented with a 3-month history of dyspnea, pleuritic pain, and nonproductive cough. Imaging demonstrated nodular infiltrates increasing toward the lung bases (Figure 1A). Bronchoscopy with lavage and transbronchial biopsies was performed to evaluate for an infectious or inflammatory etiology; however, results were unrevealing, and no infectious etiology was identified. Therefore, she underwent a video-assisted thoracic surgery biopsy that demonstrated nodular lymphoid hyperplasia with follicles centered on small airways and areas of organizing pneumonia (Figure 1B). Immunostains demonstrated follicles composed of CD3 1 T cells and CD20 1 B cells (Figure 1B) and no morphologic evidence of lymphoma or plasma cell neoplasm. Results were consistent with bronchus-associated lymphoid tissue (BALT). High-dose steroids were administered without resolution of symptoms or radiographic findings. A report of effective responses with rituximab and azathioprine in patients with common variable immunodeficiency who had a similar inflammatory lung process containing tertiary lymphoid structures, granulomas, and organizing pneumonia has been published (1). After four doses of rituximab (weekly), together with the initiation of azathioprine, her symptoms and radiographic findings improved (Figure 1C). BALT is a type of tertiary lymphoid tissue first termed by Bienenstock in 1973 (2) and described as a follicular-like aggregation of lymphoid and structural cells at airway bifurcations (3). Tertiary lymphoid tissues differ from primary (eg, thymus or bone marrow) and secondary (eg, lymph node or spleen) lymphoid tissues (3). Tertiary lymphoid tissue develops ectopically and predominately at mucosa sites and can be initiated either by autoimmunity or chronic inflammation (for review, see Reference 3). BALT formation in