Bronchus-associated lymphoid tissue in kabuki syndrome with associated hyper-IgM syndrome/common variable immunodeficiency

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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...
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