Primary Cold Agglutinin Disease

**Semantic type:** Neoplastic Process

**Definition:** A clonal B-cell lymphoproliferative disease of the bone marrow that results in autoimmune hemolytic anemia. It is distinct from lymphoplasmacytic lymphoma and IgM monoclonal gammopathy of undetermined significance. It lacks MYD88 gene mutation and is associated with trisomies of chromosomes 3, 12, and 18, and mutations in KMT2D and CARD11 genes.

**Synonyms:** - Primary CAD - Primary Cold Agglutinin-Associated Lymphoproliferative Disease - Primary Cold Agglutinin-Associated Lymphoproliferative Disorder