Can Lymphoma Produce Ascites and Pleural Effusion?
Yes, lymphoma definitively causes both pleural effusions and ascites, though pleural involvement is far more common than peritoneal involvement. 1
Epidemiology and Frequency
Pleural effusions occur in approximately 10% of all malignant pleural effusions and develop in 20-30% of patients with lymphoma (both Hodgkin's disease and non-Hodgkin's lymphoma). 1, 2 The incidence varies by study, with reports ranging from 5-33% in Hodgkin's disease and similar rates in non-Hodgkin's lymphoma. 1
Ascites is much less common than pleural effusion in lymphoma, with peritoneal cavity involvement being uncommon compared to pleural involvement. 2 However, when present, ascites represents an indication for treatment initiation in follicular lymphoma. 1
Pathophysiology: Different Mechanisms by Lymphoma Type
The mechanism differs significantly between lymphoma subtypes:
Hodgkin's disease: Effusions primarily result from obstruction of lymphatic drainage by enlarged mediastinal lymph nodes. 1, 3
Non-Hodgkin's lymphoma: Effusions occur predominantly through direct tumor infiltration of the parietal or visceral pleura. 1, 3, 4
Both types can cause chylous effusions, with non-Hodgkin's lymphoma being the most common cause of chylothorax. 1 When chylous ascites occurs, it is often accompanied by chylous pleural effusion (3 of 5 cases in one series). 5
Clinical Presentation Patterns
Pleural effusions may be unilateral or bilateral, with bilateral presentations occurring in a minority of cases. 1, 3 Dyspnea is the chief symptom in 63% of patients with lymphomatous pleural effusions. 1
The effusion characteristics include:
- Usually exudative (21/26 cases in one series) 5
- May be serous, hemorrhagic, or chylous 1
- Chylous effusions occur in approximately 5/26 cases 5
When to Initiate Treatment
According to ESMO and NCCN guidelines, the presence of ascites or pleural effusion is a specific indication to initiate systemic therapy in follicular lymphoma, even in otherwise asymptomatic patients. 1 Other indications include:
- B symptoms
- Hematopoietic impairment
- Bulky disease (≥7 cm or ≥3 nodal sites each ≥3 cm)
- Vital organ compression
- Rapid lymphoma progression 1
Diagnostic Approach
The cytologic yield is relatively poor in lymphoma compared to other malignancies, ranging from 31-55%, with the lowest yield in Hodgkin's disease. 1, 3, 6
Superior diagnostic methods include:
- Thoracoscopy with chromosome analysis: ~85% sensitivity 1, 3, 6
- Flow cytometry to demonstrate clonality: 100% sensitivity and specificity when combined with morphology 3, 2
- Immunophenotyping significantly improves diagnostic accuracy 2
Treatment and Prognosis
Systemic chemotherapy is the treatment of choice, not local pleural procedures. 1, 4 The European Respiratory Society recommends drainage before commencing chemotherapy to prevent drug accumulation and increased toxicity. 1, 4
Approximately half of patients achieve complete effusion resolution during systemic therapy. 4 Response should be assessed during the first few cycles of chemotherapy. 4
Prognostic significance is poor: Average survival after first thoracentesis is only 6-7 months. 1, 3 Patients whose effusions do not respond to systemic chemotherapy have significantly shorter survival (median 6 months) compared to responders (median >40 months). 5
Critical Pitfall to Avoid
Do not perform pleurodesis as initial management in lymphoma. Unlike other malignant effusions, lymphomatous effusions should be treated with systemic chemotherapy first, as approximately 50% will resolve with appropriate systemic therapy. 4, 5 Local treatment options (talc pleurodesis, pleuroperitoneal shunt) are reserved only for chemotherapy failures. 1