Lung diseases arising from lymphoid-related origins can include a variety of conditions characterized by lymphoid tissue proliferation or infiltration. Some notable examples are:
1. Granulomatous Diseases:
• Sarcoidosis: Characterized by noncaseating granulomas primarily involving CD4+ T cells.[1]
• Chronic Beryllium Disease: Similar to sarcoidosis, it involves noncaseating granulomas due to beryllium exposure.[2]
- lYMPHOPROLIFERATIVE DISRDERS
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Disorder
Description
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Disease Description Primary Structures Involved Spatial Category Characteristic CT Findings Follicular Bronchiolitis Reactive lymphoid hyperplasia with a peribronchiolar distribution; often associated with autoimmune diseases. Bronchioles and peribronchiolar regions Bronchioles Multiple small centrilobular nodules, patchy air trapping, and mosaic attenuation (typically mid lung fields, reflecting small airways disease). Pulmonary Lymphoid Hyperplasia Hyperplastic lymphoid tissue within the lung that may lead to respiratory symptoms; often seen with small airways involvement. Bronchioles and adjacent airway structures Bronchioles Localized or diffuse peribronchial nodules, predominantly in mid lung fields. Pulmonary MALT Lymphoma Low-grade B-cell lymphoma arising from bronchial-associated lymphoid tissue (BALT); typically indolent and often asymptomatic. Bronchial-associated lymphoid tissue (BALT) and adjacent bronchioles Bronchioles Consolidative masses or nodules with air bronchograms; often with a lower lobe predominance. Bronchiolitis Obliterans Organizing Pneumonia (BOOP/COP) Organizing pneumonia with granulation tissue filling alveolar ducts and alveoli, frequently originating from the airways. Alveolar ducts and alveolar spaces (airway origin) Alveolar Spaces Patchy peripheral consolidations and ground-glass opacities; often migratory and predominantly in peripheral or lower zones. Lymphoid Interstitial Pneumonia (LIP) Dense interstitial infiltrate of T cells, plasma cells, and histiocytes; associated with autoimmune disorders and immunodeficiency syndromes. Lung interstitium and alveolar septa Interstitium Diffuse ground-glass opacities, interlobular septal thickening, and scattered cysts; often more prominent in the lower lung zones. Nodular Lymphoid Hyperplasia (NLH) Benign lymphoproliferative disorder presenting as single or multiple nodules; usually asymptomatic. Lung parenchyma (often interstitial/peribronchial regions) Interstitium Multiple well-defined nodules, commonly seen in mid lung fields with a peribronchial distribution. Lymphomatoid Granulomatosis (LG) Rare B-cell lymphoma driven by Epstein–Barr virus; mimics pulmonary vasculitis with systemic signs. Alveoli, interstitium, and small-to-medium-sized blood vessels Interstitium Multiple nodular opacities (often with cavitation) in a peribronchovascular distribution, typically centered in the mid lung fields. Multicentric Castleman Disease (MCD) Lymphoproliferative disorder driven by HHV-8; presents with systemic symptoms (fever, lymphadenopathy) and interstitial lung involvement. Lymph nodes and lung interstitium (with extension into alveolar septa) Interstitium Diffuse interstitial thickening with ground-glass opacities and prominent lymphadenopathy, often in mid to lower zones. Posttransplant Lymphoproliferative Disorder (PTLD) Lymphoid proliferation due to immunosuppression after transplantation; can involve lung parenchyma. Lung parenchyma including alveoli and interstitium Interstitium Multifocal nodules and consolidations with a random distribution across upper and lower lung fields. AIDS-related Lymphoma (ARL) Lymphoma associated with AIDS that primarily affects lung parenchyma. Lung parenchyma and mediastinal/hilar lymph nodes Interstitium Solitary or multiple pulmonary nodules/masses with associated mediastinal/hilar lymphadenopathy; distribution is usually non-zonal. Intravascular Lymphoma Rare lymphoma characterized by proliferation of lymphoid cells within blood vessels; may involve lung parenchyma. Pulmonary blood vessels embedded in the lung interstitium Interstitium Diffuse, subtle interstitial infiltrates with small nodular opacities, typically noted in the mid lung fields. IgG4-Related Lung Disease A systemic condition that causes interstitial pneumonia and lymphoid hyperplasia; involves bronchovascular and lymphatic structures. Bronchovascular bundles and lung interstitium Interstitium Diffuse interstitial thickening, ground-glass opacities, and bronchovascular bundle thickening, most often in the mid lung zones. Non-Hodgkin Lymphoma (NHL) Primary pulmonary lymphoma that arises directly within lung tissue, typically manifesting as nodules or masses. Lung parenchyma (commonly the interstitium) Interstitium Solitary or multiple masses/nodules with consolidative features; frequently with a lower lobe predominance. Hodgkin Lymphoma Although primarily a lymphatic malignancy, it can involve the lungs; typically presenting with lymphadenopathy and pulmonary infiltrates. Mediastinal/hilar lymph nodes and lung parenchyma Interstitium Prominent mediastinal and hilar lymphadenopathy with associated pulmonary infiltrates; distribution may vary. Sarcoidosis Granulomatous disease with noncaseating granulomas; often presents with enlarged hilar/mediastinal nodes and interstitial lung involvement. Lung interstitium and alveolar septa; mediastinal/hilar lymph nodes Interstitium Bilateral hilar and mediastinal lymphadenopathy with a perilymphatic distribution of micronodules, typically with an upper lobe predominance. Primary Effusion Lymphoma (PEL) HHV-8–driven lymphoma presenting as a lymphocytic-exudative pleural effusion without an associated parenchymal mass, typically in immunocompromised patients. Pleural space Pleura Unilateral or bilateral pleural effusions without a discernible parenchymal mass; sometimes with mild pleural thickening. This updated table now includes Primary Effusion Lymphoma (PEL) along with its unique clinical and imaging characteristics, organized spatially and with specific CT findings for each condition.
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