AJHE2312 - CME/CMLE - The Histopathology of Cold Agglutinin Disease–Associated B-Cell Lymphoproliferative Disease-The Histopathology of Cold Agglutinin Disease–Associated B-Cell Lymphoproliferative Disease

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

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The article discusses Cold Agglutinin Disease (CAD) as a novel B-cell lymphoproliferative disorder with secondary morbidity mainly associated with anemia and Raynaud phenomenon. CAD is characterized by intraparenchymatous aggregates of small B cells in the bone marrow with mutations in KMT2D, CARD11, and CXCR4 genes. The disease has been identified as a separate entity in recent classifications of mature B-cell neoplasms. The clinical and laboratory diagnosis of CAD entails laboratory tests to detect cold agglutinins, direct antiglobulin tests, and exclusion of other underlying conditions. Histopathological examination reveals nodular lymphoid infiltrates in the bone marrow, predominantly composed of small B cells. Genetic analysis shows recurrent mutations in KMT2D, CARD11, and CXCR4 genes. Distinct genetic and molecular profiles differentiate CAD from other small B-cell lymphomas. Treatment strategies focus on managing anemia in symptomatic patients by measures like avoiding cold exposure and utilizing therapies like rituximab. The article emphasizes the importance of recognizing the pathology features of CAD to differentiate it from other diseases, particularly lymphoplasmacytic lymphoma and marginal zone lymphoma. References to relevant studies and guidelines are provided to support the information presented in the review.

Keywords

Cold Agglutinin Disease

CAD

B-cell lymphoproliferative disorder

anemia

Raynaud phenomenon

KMT2D gene

CARD11 gene

CXCR4 gene

histopathological examination

rituximab