title: Acquired immune-mediated thrombophilia in lymphoproliferative disorders.
abstract: We analyzed 66 cases of immune-mediated thrombophilia in patients with lymphoma reported in the literature. Sixty-one cases had a lupus anticoagulant, three an antibody to protein S, one to protein C, and one to ADAMTS 13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). Lupus anticoagulants occurred in all histological subtypes of non-Hodgkin lymphoma, except mantle cell lymphoma, MALT (mucosa-associated lymphoid tissue) lymphoma, and angioimmunoblastic T-cell lymphoma, and rarely in Hodgkin lymphoma. The largest number of cases was described in splenic marginal zone and lymphoplasmacytic lymphoma. Lupus anticoagulants were highly associated with immunoglobulin M (IgM) paraproteinemia, autoimmune hemolytic anemia, and immune thrombocytopenia. About half of the patients had thrombotic events (antiphospholipid antibody syndrome). Venous thromboembolism was more than twice as common as arterial thrombosis; 6.5% had a catastrophic antiphospholipid antibody syndrome. The lupus anticoagulant could be eliminated by lymphoma treatment (chemoimmunotherapy or splenectomy) in more than one-third of patients. It is suggested that a search for lupus anticoagulant should be done in all patients with lymphoma, because a diagnosis of lupus anticoagulant may influence the management of lymphomas in some patients.
authors.affiliation: Division of Hematology and Hemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria. klaus.lechner@meduniwien.ac.at
authors.forename: Klaus
authors.initials: K
authors.lastname: Lechner
authors.forename: Ingrid
authors.initials: I
authors.lastname: Simonitsch
authors.forename: Johanna
authors.initials: J
authors.lastname: Haselböck
authors.forename: Ulrich
authors.initials: U
authors.lastname: Jäger
authors.forename: Ingrid
authors.initials: I
authors.lastname: Pabinger
mesh_terms: D051722:ADAM Proteins / Q000276:immunology; D000071120:ADAMTS13 Protein; D001323:Autoantibodies / Q000097:blood; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000097:blood*; D008223:Lymphoma / Q000276:immunology*; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000276:immunology*; D011486:Protein C / Q000276:immunology; D017293:Protein S / Q000276:immunology; D019851:Thrombophilia / Q000209:etiology / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D016682:Lupus Coagulation Inhibitor; D011486:Protein C; D017293:Protein S; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human