Chronic lymphocytic leukemia/small lymphocytic lymphoma involving the aortic valve.

Chronic lymphocytic leukemia/small lymphocytic lymphoma is a neoplasm composed of monomorphic small B lymphocytes in the peripheral blood, bone marrow, spleen, and lymph nodes, forming proliferation centers in tissue infiltrates (Muller-Hermelink HK, Montserrat E, Catovsky D, et al. Chronic lymphocytic leukaemia/small lymphocytic lymphoma, in Swerdlow SH (ed). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France, International Agency for Research on Cancer, 2008, pp. 180-182). We report a case of a 77-year-old man with a medical history of chronic lymphocytic leukemia who presented with worsening chest pain over 8 weeks. Imaging studies revealed severe aortic stenosis and moderate mitral regurgitation. He subsequently underwent minimally invasive aortic valve replacement and mitral repair at our institution. Grossly, the specimen consisted of a trileaflet valve with multiple yellow-white focally hemorrhagic and calcified nodules over its surface. Histologically, a lymphocytic infiltrate composed of monotonous small cells with scant cytoplasm was seen as well as calcification and fibrosis. Immunohistochemical stains were positive for CD20, PAX5, CD5, and CD23. To our knowledge, this is the first reported case of an immunohistochemically documented chronic lymphocytic leukemia/small lymphocytic lymphoma to involve a cardiac valve.