Hemostatic complications associated with paraproteinemias.

Hemorrhagic events are a common complication at diagnosis, during treatment, and with disease progression of lymphoproliferative disorders that produce monoclonal immunoglobulins or paraproteins. Typical causes of bleeding include vascular infiltration by malignant cells, hyperviscosity syndrome, thrombocytopenia secondary to bone marrow infiltration or treatment, uremia, and coagulopathies secondary to liver dysfunction or sepsis. However, there are several unique hemostatic derangements associated with lymphoproliferative disorders that express paraproteins and these are the subject of this article (eg, inhibition of fibrin polymerization, qualitative platelet dysfunction, acquired von Willebrand factor deficiency, heparin-like circulating anticoagulants, light chain amyloid associated hemostatic disorders, acquired coagulation factor deficiencies, and acquired hypercoagulable states). The exact role that paraproteins play in many of these conditions is unknown or incompletely investigated. Often, abnormal hemostasis test results are not accompanied by clinically apparent hemostatic complications. Management of paraprotein-related hemostatic complications varies in strategy and efficacy, depending on the underlying mechanism and type of lymphoproliferative disease.