FACTOR-V INHIBITOR ASSOCIATED WITH COLD AGGLUTININ DISEASE

An 81-year-old man known to have a stable cold agglutinin syndrome pre sented with a progressive cerebral hemorrhage. Coagulation tests revea led prolonged APTT and prothrombin time and severely decreased factor V activity, which could not be normalized by mixing with normal plasma . The patient appeared refractory to substitution with fresh-frozen pl asma, suggesting the presence of a circulating inhibitor specific for factor V. To our knowledge, this is the first case of a lymphoprolifer ative disease leading to a cold agglutinin syndrome and a putative inh ibitor of factor V. In patients with paraproteinemia presenting with b leeding diathesis, the presence of a circulating inhibitor of a specif ic coagulation factor must be considered.