Primary cold agglutinin disease: a recently recognised diagnostic entity in WHO and ICC.

The disease is linked to an underlying BM-based clonal B-cell proliferation.[[3]] Cases associated with overt lymphoma, in particular lymphoplasmacytic lymphoma (LPL), or infections are excluded from the pCAD category and are considered secondary cold agglutinin syndromes. PCAD diagnosis needs appropriate correlation of clinical and laboratory findings with bone marrow histology and molecular data. When clinical and laboratory findings are suggestive of pCAD and BM histology shows a low-grade clonal B-cell infiltrate, the mutational analysis of I MYD88 i status is mandatory to exclude an LPL. [Extracted from the article]