Pyothorax-associated lymphoma (PAL) is a lymphoproliferative disorder developing in the pleural cavity after a long-standing history of pyothorax. The pathogenesis, clinical features and optimal treatment have not been clarified. To investigate clinicopathological features of pyothorax-associated lymphoma (PAL), we examined medical records of 98 patients (88 males and 10 females) with PAL at a median age of 70 (range, 51–86). Seventy-nine patients had a history of artificial pneumothorax. Median interval between diagnosis and artificial pneumothorax was 43 years (range, 19–64). At diagnosis, performance status was 0–1 (n=56) and 2–4 (n=42). Clinical stages were I (n=42), II (n=26), III (n=8) and IV (n=22). In situ hybridization using a probe for EBV-encoded RNA-1 demonstrated the presence of the EBV genome in the nucleus of tumor cells in 28 of 29 (88%) patients. Immunohistochemical studies revealed that 12 of 14 (86%) and 11 of 17 (65%) were positive for EBNA-2 and LMP-1, respectively. Seventeen were treated supportively. The other 81 received aggressive treatments; chemotherapy (n=52), radiotherapy (n=7), surgery (n=4) and combination (n=18). Response rates to chemotherapy, radiotherapy, and chemoradiotherapy were 56%, 71%, and 83%, respectively. Five-year overall survival was 0.35 (95% confidence interval, 24–45%). The 5-year overall survival rates were 0.47 (95% CI, 0.30– 0.63), 0.35 (95% CI, 0.16– 0.54) and 0.15 (95% CI, 0.04–0.33) in patients with stages I, II and I-IV disease, respectively (Figure 1). Overall survival are different among the three groups (p <0.0001). Causes of deaths were PAL (n=39), respiratory failure (n=13), and others (n=12). Multivariate analysis identified prognostic factors for overall survival; lactate dehydrogenase levels (hazard ratio (HR)=2.36, p=0.013), sex (female vs. male) (HR=0.15, p=0.01), performance status (2–4 vs. 0–1) (HR=2.20, p=0.02), clinical stages (III/IV vs. I/II) (HR=1.95, p=0.037) and chemotherapy (HR=0.31, p=0.01). Most patients with PAL are elderly, and have comorbidities, while some of them achieve durable remission with appropriate treatments. These findings prompt us to establish an optimal treatment strategy based on risk stratification of individual patients.

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Topics:
lymphoma, pleural empyema, chemotherapy regimen, pneumothorax, artificial, radiation therapy, borderline neoplasms, disease remission, epstein-barr virus nuclear antigens, lactate dehydrogenase, lymphoproliferative disorders

Disclosure: No relevant conflicts of interest to declare.

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2006, The American Society of Hematology
2006
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