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.