Pulmonary lymphomatoid granulomatosis
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
Pulmonary lymphomatoid granulomatosis (PLG) is an uncommon pulmonary disorder characterized by multiple pulmonary nodular lesions with lymphocytic invasion of vascular walls on biopsy. Averill Liebow and colleagues first described PLG as a clinicopathologic entity in 1972 . Since that time, the clinical implications of this lesion have remained controversial, as evidenced by a long list of competing synonyms including angiocentric immunoproliferative lesion and angiocentric lymphoma [2-4]. Some have argued that the histopathologic pattern of lymphomatoid granulomatosis, which can occur in pulmonary and extrapulmonary tissue, is a nonspecific manifestation of diverse pathogenetic conditions, including autoimmunity, infection, and malignancy .
It is generally believed that PLG represents a form of lymphoproliferative disorder. The World Health Organization (WHO) classification scheme places lymphomatoid granulomatosis under the generic heading of B cell proliferations of uncertain malignant potential . These lymphoproliferative disorders are in the family of Epstein-Barr virus (EBV)-associated B cell lymphomas [5-8]. (See "Clinical manifestations and treatment of Epstein-Barr virus infection", section on 'Lymphoproliferative disorders' and "Epidemiology, clinical manifestations, pathologic features, and diagnosis of diffuse large B cell lymphoma", section on 'Lymphomatoid granulomatosis'.)
In addition to PLG, the lung is the primary organ of involvement in a spectrum of lymphocytic and lymphoproliferative disorders. These include lymphoid interstitial pneumonia, plasma cell interstitial pneumonia, angioimmunoblastic lymphadenopathy, and primary pulmonary lymphoma [9,10]. Pulmonary lymphomatoid granulomatosis will be reviewed here. Clinical aspects of other pulmonary lymphocytic and lymphoproliferative disorders are discussed separately. (See "Lymphoid interstitial pneumonia in adults" and "Inflammatory myofibroblastic tumor (plasma cell granuloma) of the lung" and "Clinical manifestations, pathologic features, and diagnosis of peripheral T cell lymphoma, not otherwise specified", section on 'Angioimmunoblastic T cell lymphoma' and "Clinical manifestations, pathologic features, and diagnosis of extranodal (MALT) and nodal marginal zone lymphomas".)
Pulmonary lymphomatoid granulomatosis generally presents between the ages of 30 and 50, although patients can be affected at any age [4,11-17]. It is predominantly seen in men, with an estimated male to female ratio of at least 2:1 [5,18]. The effects of race and geography on disease incidence are not known, although a higher diagnosis rate is reported in Western countries .
Pulmonary lymphomatoid granulomatosis appears to be associated with Epstein-Barr virus (EBV) infection in most if not all cases [5,6]. A small number of cases show no demonstrable evidence of EBV infection, and may represent a distinct form of peripheral T cell lymphoma . Alternatively, these cases may reflect grade 1 disease with infrequent EBV-positive cells that were not detected [19-21]. (See 'Grading' below and "Epidemiology, clinical manifestations, pathologic features, and diagnosis of diffuse large B cell lymphoma", section on 'Lymphomatoid granulomatosis'.)
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- CLINICAL PRESENTATION
- Symptoms and signs
- Physical examination
- Laboratory studies
- Lung function testing
- Imaging studies
- Genetic features
- DIFFERENTIAL DIAGNOSIS
- NATURAL HISTORY AND PROGNOSIS
- SUMMARY AND RECOMMENDATIONS