INTRODUCTION: Eventhough pleural involvement by non-Hodgkin’s lymphoma is common, primary pleural lymphomas are rare. There are two types of primary pleural lymphomas described: body cavity based lymphoma in patients who have HIV and, pyothorax associated lymphoma (PAL) which has been described more commonly in patients with a history of tuberculosis and pleural infections. We describe a case of pyothorax associated lymphoma presenting with severe bronchiectasis and a bloody pleural effusion.
CASE PRESENTATION: A 66 year old female who presents with fever and cough. Chest radiograph (CXR) showed a right upper lobe abnormality which led to a diagnosis of community acquired pneumonia. She was treated with oral antibiotics with improvement of her symptoms. Given persistent changes on CXR, she had a chest computed tomography (CT) done. Findings were consistent with severe focal bronchiectasis and collapse of the right upper and right middle lobes with a minimal amount of pleural fluid. The patient reported an episode of whooping cough at age six and an episode of pneumonia 30 years prior to presentation; she denied a history of exposure to tuberculosis and had previous negative purified protein derivative (PPD) tests. The patient remained symptom free for two years, until she experienced another similar episode of fever and cough. Oral azithromycin improved her symptoms, however, the cough persisted. Shortly after, she developed chest pressure and a dull pain over her left hemithorax, along with dyspnea to moderate exertion. CT chest revealed an extensive right-sided pleural effusion. She denied fever, diaphoresis, weight loss or excessive fatigue. Thoracentesis was done with removal of 400ml of bloody fluid. This led to a diagnosis of large cell lymphoma with morphologic and immunophenotypic features consistent with pyothorax-associated lymphoma (PAL). The patient is currently undergoing chemotherapy with rituximab, etoposide, cyclophospamide, doxorubicin, vincristine and prednisone (R-EPOCH).
DISCUSSION: Pyothorax associated lymphoma was first described in 1987 by Aosaza and colleagues in 2.2% of patients with chronic pyothorax (CP) in Japan, it is a non-Hodgkin’s lymphoma (NHL) most often comprised only of B-cells developing in the pleural cavity of patients with a chronic inflammatory process within the pleural space. Patients typically presented with a 20 to 64 year history of pyothorax caused by artificial pneumothorax performed for the treatment of pulmonary or pleural tuberculosis. Cases of PAL were later described in the Western hemisphere. Most common symptoms included back or chest pain and fever (43%), most patients (97%) had a tumor mass. Response to chemotherapy was similar to other B-cell lymphomas and the estimated 1-, 3-, and 5-year survival rates were 48.6%, 27.0%, and 21.6%, respectively. Survival was better for 57% of patients showing complete response to chemotherapy (ie, 88.2%, 63.0%, and 49.0%, respectively). The EBV genome was present in 70% of cases (1). It is now recognized that PAL is associated to any form of chronic inflammation and cases without a prior pyothorax have been described; prognosis remains guarded. PAL may have common underlying factors with mucosal associated lymphomas and thyroid lymphomas, in which chronic inflammation caused by Helicobacter pylori and Hashimoto’s thyroiditis respectively, play an important role in causality (2).
CONCLUSIONS: Pyothorax associated lymphoma is an aggressive and rare primary pleural lymphoma that is related to chronic inflammation within the pleural space. Our patient presented with chronic bronchiectasis and a bloody pleural effusion. Pyothorax associated lymphoma should be kept in the differential diagnosis of any chronic pleural effusion or pleural based mass.
Reference #1 Nakatsuka S, Masayuki Y, Yoshihiko H, Satoru Y, Keiji I, and Katsuyuki A. J Clin Oncol 20:4255-4260.
Reference #2 Giardino A, O’Regan KN, Hargreaves J, Jagannathan J, Park D, Ramaiya N, Fisher D. J Clin Oncol 29, 2011 (Published ahead of print)
DISCLOSURE: The following authors have nothing to disclose: Andres Sosa, Renelle Myers, German Pihan, Gaetane Michaud
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