The Histopathology of Cold Agglutinin Disease-Associated B-Cell Lymphoproliferative Disease

Abstract

Objectives: Primary cold agglutinin disease is a type of autoimmune hemolytic anemia caused by circulating antibodies against I antigen, a carbohydrate expressed on most cells, including red blood cells. The underlying disease has been characterized in recent years as a distinct B-cell lymphoproliferative disease of the bone marrow, occurring mostly in the elderly. The disease has been now been included as a separate entity in the most recent classifications of mature B-cell neoplasms.

Methods: A review of the characteristics of cold agglutinin disease is provided, with an emphasis on the pathology features.

Results: A detailed description of the histopathology, immunophenotype, and genetics of cold agglutinin disease is provided and compared to other B-cell lymphoproliferative diseases in the bone marrow with similar features.

Conclusions: Recognition of the pathology features of cold agglutinin disease allows to distinguish it from other diseases, especially lymphoplasmacytic lymphoma and marginal zone lymphoma.

Keywords: Autoimmune; Cold agglutinin disease; Diagnosis; Hemolytic anemia; Lymphoma.