A 53-year-old man, who had initially presented with an upper respiratory tract infection, was found to have a stage IV follicular low-grade lymphoma with malignant cells that were positive for CD19, CD20, CD10, surface kappa, and CD45 and negative for CD5. A grade 1 tumor (follicular center cell lymphoma/follicular small cleaved) was suspected. Enlarged lymph nodes (2.5 to 4.0 cm) were found in the mediastinum, azygoesophageal recess, and periaortic region as well as in the porta hepatis, peripancreatic, mesenteric, and celiac regions.

After the patient received his first cycle of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy, a chest film showed a right-sided pleural effusion (A). Thoracentesis was performed. The pleural fluid protein level was 5.0 g/dL, and the lactate dehydrogenase level was 124 U/L. The triglyceride level was elevated (2800 mg/dL). Flow cytometry of the pleural effusion showed no evidence of a monoclonal B-cell process. The patient required ultrasound-guided thoracentesis for each of the next 3 cycles of CHOP chemotherapy; the chylous pleural fluid obtained at thoracentesis is shown (B). He then underwent a right thoracoscopy, mini-thoracotomy with ligation of the thoracic duct, and doxycycline talc pleurodesis. There were no further pleural effusions.

Pleural effusions associated with lymphomas are usually exudative and are associated with poor outcomes.1-4 They are rarely chylous. The mechanism of chylothorax is likely lymphoma-related eventration of the thoracic duct.5 Various treatments have been used, including systemic chemotherapy, surgical ligation of the thoracic duct, octreotide, chemical pleurodesis, and radiation therapy.6-8 There is no standard management of this uncommon manifestation of non-Hodgkin lymphoma, and treatment must be individualized.

 

This patient's disease recurred within 4 months of completing chemotherapy. He received 2 cycles of dexamethasone, high-dose cytarabine, and cisplatin (DHAP) chemotherapy but refused high-dose chemotherapy with autologous stem cell support. He died 14 months after diagnosis.