Small lymphocytic lymphoma that developed in a patient with long-standing cold agglutinin disease / 대한내과학회지

ABSTRACT

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia (AIHA) induced by cold antibodies. CAD constitutes 16~32% of AIHA cases and the causative cold autoantibodies are commonly specific against blood group I or i antigens. CAD is closely related to lymphoid neoplasia and infections such as Mycoplasma and Epstein-Barr virus (EBV). Primary CAD has a tendency to develop into malignant lymphoma over its course. We experienced a case of small lymphocytic lymphoma during the treatment of primary CAD over 11 years. The patient was a 67-year-old man who complained of a neck mass. Node biopsy revealed diffuse neoplastic proliferation of small monotonous lymphoid cells with polygonal or oval nuclei;these cells were CD20- and CD5-positive. Follow-up chest and neck computed tomography after treatment with chlorambucil and prednisolone showed partial resolution and stable disease status for over 2 years. We report a case of small lymphocytic lymphoma that developed in a patient with long-standing cold agglutinin disease for 11 years and review the literature.