Primary pulmonary lymphoma

Primary pulmonary lymphoma (PPL) is a rare form of non-Hodgkin lymphoma that originates in the lung parenchyma without evidence of extrapulmonary involvement at the time of diagnosis or within three months thereafter. It accounts for less than 0.5% of all primary pulmonary malignancies and approximately 3-4% of extranodal lymphomas. The most common subtype is mucosa-associated lymphoid tissue (MALT) lymphoma, also known as extranodal marginal zone B-cell lymphoma, which comprises the majority of cases. Less common subtypes include diffuse large B-cell lymphoma and lymphomatoid granulomatosis. The disease primarily affects the respiratory system. Many patients are asymptomatic at diagnosis, with the condition discovered incidentally on chest imaging. When symptoms are present, they may include chronic cough, dyspnea (shortness of breath), chest pain, hemoptysis (coughing up blood), and constitutional symptoms such as fever, night sweats, and unintentional weight loss. Radiographic findings typically show solitary or multiple pulmonary nodules, consolidation, or ground-glass opacities. Some patients may have an underlying autoimmune condition, chronic infection, or immunodeficiency that predisposes them to the development of pulmonary lymphoma. Diagnosis requires tissue biopsy, often obtained through bronchoscopy or surgical lung biopsy, with histopathological and immunohistochemical confirmation. Treatment depends on the subtype and extent of disease. Low-grade MALT lymphomas often have an indolent course and may be managed with observation, surgical resection, or localized radiation therapy. Chemotherapy, immunotherapy (such as rituximab), or chemoimmunotherapy may be employed for more aggressive subtypes like diffuse large B-cell lymphoma. The prognosis for MALT-type PPL is generally favorable, with five-year survival rates exceeding 80%, while high-grade subtypes carry a less favorable prognosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Primary pulmonary lymphoma