Atypical Presentation of Small Lymphocytic Lymphoma with Pericardial Effusion

Abstract

BACKGROUND Small lymphocytic lymphoma (SLL) is a low-grade B-cell non-Hodgkin lymphoma and is the solid tumor equivalent of chronic lymphocytic leukemia (CLL) that is found in the peripheral blood. SLL typically presents with lymphadenopathy and is rarely associated with cardiac involvement. This report is of a case of lymphomatous pericardial effusion in a 61-year-old woman who presented with dyspnea. CASE REPORT A 61-year-old woman presented to the emergency department with a three-month history of worsening shortness of breath on exertion. Her symptoms progressed to shortness of breath at rest, with night sweats and chills. She had no weight loss. She was found to have a pericardial effusion, and an urgent pericardiocentesis was performed to prevent cardiac tamponade. Analysis of the pericardial fluid was consistent with a diagnosis of SLL. A bone marrow biopsy and a biopsy of a renal mass were consistent with a diagnosis of SLL. She was treated with rituximab and bendamustine with granulocyte-colony stimulating factor (G-CSF) support and was discharged home. CONCLUSIONS A case is presented of a rare association between SLL and pericardial effusion with a favorable outcome following urgent pericardiocentesis to prevent cardiac tamponade followed by chemotherapy.

Figure 1.

(A, B) Computed tomography (CT) angiography of the chest shows a moderate to large pericardial effusion (arrows).

Figure 2.

(A, B) Computed tomography (CT) of the abdomen and pelvis shows multiple solid and cystic lesions, some pf which appear heterogeneous (arrows).

Figure 3.

Immunohistochemical staining shows that the lymphoma cells are positive for CD79a, PAX-5, and CD5, and negative for CD3, Bcl-6, Bcl-2, CD20, and Bcl-1, with a low cell proliferation rate on immunostaining for Ki67. Immunohistochemistry for the proliferation index shows that 1–2% of the lymphoma cells are Ki-67 positive. The cell morphology, immunohistochemistry, and flow cytometry findings support the diagnosis of a CD5-positive small lymphocytic lymphoma (SLL), which is a low-grade, B-cell, non-Hodgkin lymphoma (NHL).