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Pyothorax-Associated Lymphoma: A Review of 106 Cases

From the Department of Pathology, Osaka University Graduate School of Medicine, Suita; and Pathology and Surgery Sections, National Kinki-Chuo Hospital, Sakai, Osaka, Japan.

Address reprint requests to Katsuyuki Aozasa, MD, Department of Pathology (C3), Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan; email: aozasa{at}molpath.med.osaka-u.ac.jp

PURPOSE: Pyothorax-associated lymphoma (PAL) is a non-Hodgkin’s lymphoma developing in the pleural cavity after a long-standing history of pyothorax. Full details of PAL are provided here.

PATIENTS AND METHODS: Clinical and pathologic findings were reviewed in 106 patients with PAL collected through a nationwide survey in Japan.

RESULTS: Age of the patients with PAL was 46 to 82 years (median, 64 years), with a male/female ratio of 12.3:1. All patients had a 20- to 64-year (median, 37-year) history of pyothorax resulting from artificial pneumothorax for treatment of pulmonary tuberculosis (80%) or tuberculous pleuritis (17%). The most common symptoms on admission were chest and/or back pain (57%) and fever (43%). Laboratory data showed that the serum neuron-specific enolase level was occasionally elevated (3.55 to 168.7 ng/mL; median, 18.65 ng/mL), suggesting a possible diagnosis of small-cell lung cancer. Histologically, PAL usually showed a diffuse proliferation of large cells of B-cell type (88%). In situ hybridization study showed that PAL in 70% of the patients was Epstein-Barr virus (EBV)-positive. PAL was responsive to chemotherapy, but the overall prognosis was poor, with a 5-year survival of 21.6%.

CONCLUSION: This study established the distinct nature of PAL as a disease entity. PAL is a non-Hodgkin’s lymphoma of exclusively B-cell phenotype in the pleural cavity of patients with long-standing history of pyothorax, and is strongly associated with EBV infection. Development of PAL is closely related to antecedent chronic inflammatory condition; therefore, PAL should be defined as malignant lymphoma developing in chronic inflammation.

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