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Primary Effusion Lymphoma in a non-HIV Patient: a Case Report and Discussion of Treatment Modalities

Medical University of South Carolina, Charleston, SC

INTRODUCTION: Pleural effusions from lymphoma occur from mediastinal obstruction, chylothorax, and from lymphomatous infiltration of the pleura. Rarely, lymphoma occurs in the pleural space as a presenting manifestation. There is a subset of lymphoma based in serosal surfaces originally described by Knowles in 1989 that is almost universally associated with advanced HIV infection[1]. We describe a case of primary pleural SCLL that proved refractory to pleuridesis.

CASE PRESENTATION: The patient is a 63 year-old with a two-week history of dyspnea on exertion. Chest x-ray revealed large bilateral pleural effusions without cardiomegaly. CT scan of chest revealed no mediastinal adenopathy, but there was slight bilateral pleural thickening. Thoracentesis on both effusions revealed sanguinous, lymphocytic exudates. Flow cytometry revealed 86% lymphocytes with a 10% clonal population of B cells with kappa light chain rearrangement. Bone marrow biopsy revealed an identical small abnormal B-cell clone. Pleurodesis on the left was attempted but failed and his pleural effusions reaccumulated. A permanent, tunneled, indwelling catheter (Pleurx®) was placed in the left pleural space and provided some symptomatic relief; however, his effusions persisted. With initiation of chemotherapy, his effusions have decreased in size and his dyspnea has abated.

DISCUSSIONS: There are subsets of B-cell lymphoma known as primary pleural lymphomas (PPL) that manifest as an isolated pleural effusion(s). There are distinct subtypes with different phenotypes and clinical courses. Arvanitakis e.a. definitively identified the causative agent of pleural effusion lymphoma (PEL) in advanced AIDS patients as KSHV (now Human Herpes virus 8) [2]. Primary pleural lymphoma has two distinct subtypes in non-HIV infected patients. There are those patients who have had longstanding pleural inflammation (mainly patients with chronic pleural tuberculosis.)[3]. The other subgroup of non-HIV patients with PPL are usually elderly and HHV-8 has been implicated. Prognosis in HIV associated PEL is grim. The patients with non-HIV associated PPL achieve remission of their disease with chemotherapy[3]. Because survival with malignant effusion is usually less than six months, treatment focuses on alleviation of dyspnea and minimization of hospitalization. For many malignant effusions, a tunneled, indwelling pleural catheter has proved as efficacious as pleurodesis. However, patients with malignant effusions from breast cancer and lymphoma can have prolonged survival. Therefore, they are at increased risk for complications from the catheter. Thus, the role of the indwelling catheter is not as well established in patients with these malignancies. Other cases of PEL reported in the literature have shown good survival and response rates to systemic chemotherapy[3].

CONCLUSION: Primary pleural lymphoma should be considered in the differential diagnosis of all patients with lymphocytic pleural effusions without adenopathy. In non-HIV primary pleural lymphoma, remission rates are excellent. Therefore, treatment should not be viewed as palliative as in other malignant effusions. The primary treatment modality of primary pleural lymphoma in both HIV and non-HIV patients is systemic chemotherapy. Use of tunneled, indwelling pleural catheter (pleurx) or pleurodesis can be used as adjunctive therapy for symptom control when chemotherapy has failed.

DISCLOSURE: J.T. Heidecker, None.

REFERENCES

1. Knowles, D. et al. Molecular genetic analysis of three AIDS-associated neoplasms of uncertain lineage demonstrates their B-cell derivation and the possible pathogenetic role of Epstein-Barr virus. Blood,1989 .73 : p.792 –799.
2. Arvanitakis, E. et al. Establishment and Characterization of a Primary Effusion (Body Cavity-Based) Lymphoma Cell Line (BC-3) Harboring Kaposi’s Sarcoma-Associated Herpesvirus (KSHV/HHV-8) in the Absence of Epstein-Barr Virus. Blood,1996 .88(7) : p.2648 –2654.
3. Martin, A. Epstein-Barr virus-associated primary malignant lymphomas of the pleural cavity occuring in longstanding pleural chronic inflammation. Human Pathology,1994 .25(12) : p.14 –18.