Cold agglutinin disease

Cold agglutinin disease is a type of autoimmune hemolytic anemia (see this term) defined by the presence of cold autoantibodies (autoantibodies which are active at temperatures below 30°C).

Paraproteinemia

An abnormal immunoglobulin or part of an Ig (light chain) in the circulation. Paraproteins are typically produced by a clonal population of B-cell derived plasma cells.


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PMID (PMCID)
9486926
 MALE
Factor V inhibitor associated with cold agglutinin disease.
van Spronsen DJ, Oosting JD, Hoffmann JJ, Breed WP.
Ann Hematol. 1998;76(1):49-50.
The patient appeared refractory to substitution with fresh-frozen plasma, suggesting the presence of a circulating inhibitor specific for factor V. To our knowledge, this is the first case of a lymphoproliferative disease leading to a cold agglutinin syndrome and a putative inhibitor of factor V. In patients with paraproteinemia presenting with bleeding diathesis, the presence of a circulating inhibitor of a specific coagulation factor must be considered.