Primary Pulmonary Follicular Lymphoma - a Rare Presentation of Non-Hodgkin Lymphoma

INTRODUCTION

Primary pulmonary lymphoma (PPL) is an uncommon form of non-Hodgkin lymphoma that begins in the lungs. PPL originates directly within the lung tissue, unlike more prevalent lymphomas that frequently metastasize to the lungs from other sites. Lung primary non-Hodgkin lymphoma (NHL) is an exceptionally uncommon condition, representing approximately 0.4% of all malignant lymphomas. Only 3-4% of non-Hodgkin lymphoma (NHL) that does not start in lymph nodes is primary pulmonary lymphoma (PPL). This is less than 1% of all NHL cases and 0.5-1% of all primary lung cancers. Here we present a rare case of primary pulmonary follicular lymphoma characterized by bilateral pleural effusion.

AIM

The main objective in managing primary pulmonary lymphoma (PPL) is to promptly identify, precisely diagnose, and efficiently treat the condition to enhance patient outcomes. This entails increasing knowledge, employing sophisticated diagnostic methods, precisely determining the stage of the disease, and creating personalized treatment strategies.

CASE REPORT

A 74-year-old male patient presented with shortness of breath that was progressively worsening for 3 to 4 weeks. The patient denied having fever, chills, sweating, unexpected weight loss, or lymphadenopathy in the armpit, neck, or groin. The patient had no prior history of lung disease, and he did not use home oxygen or inhalers. The patient smoked up to 1 pack of cigarettes daily for 10 years but quit in 1995. He was initially treated with antibiotics, but the dyspnea persisted. A chest X-ray and CT angiogram were done at the time, which displayed bilateral pleural effusions, moderate on the right and small on the left, and no evidence of pulmonary thromboembolic disease. Therapeutic and diagnostic aspirations had been made. The cytology of the fluid was suggestive of exudative and found negative for malignant cells. Despite initial negative cytology for malignant cells, the patient returned 2 weeks later with dyspnea and increased bilateral pleural effusions from prior studies. A second thoracocentesis and analysis of the fluid, using flow cytometry and FISH, showed a single group of B cells that were positive for lambda CD10. This is a sign of follicular lymphoma, which is supported by changes in BCL6, BCL2, and t(14:18)/IGH-BCL2.

CONCLUSION

In summary, this case adds to the existing knowledge about the various ways NHL can present and supports the need for a comprehensive approach to caring for patients with pleural effusion. It also highlights the importance of early detection and prompt initiation of treatment for unexpected cancer cases, improving results through focused therapeutic approaches.

No relevant conflicts of interest to declare.