fibrin and Paraproteinemias

Topics: Factor XIII; Factor XIII Deficiency; Female; Fibrin; Humans; Middle Aged; Paraproteinemias

Topics: Acyclovir; Antiviral Agents; Blood Coagulation Disorders; Female; Fibrin; Herpes Zoster; Humans; Hyperlipidemias; Infarction, Middle Cerebral Artery; Lymphangiectasis, Intestinal; Middle Aged; Paraproteinemias; Protein S; Protein-Losing Enteropathies; Urinary Tract Infections

A 66-year-old man with peptic ulcer disease developed a paraprotein that resulted in a spontaneously prolonged prothrombin time, activated partial thromboplastin time, and thrombin clotting time. Although the reptilase time was normal, the thrombin clotting time failed to correct with the addition of normal plasma, calcium, or protamine sulfate. The patient's purified fibrinogen was normal, but his serum contained an IgG that inhibited the clotting of normal plasma and purified fibrinogen in the presence of thrombin. In contrast to previously described paraproteins, this patient's IgG appeared to inhibit the activity of thrombin per se rather than to interfere with fibrinogen cleavage or fibrin polymerization. Although immunoprecipitation between thrombin and the paraprotein could not be demonstrated, the patient's purified IgG, in the presence of thrombin, decreased the thrombin activity on a chromogenic substrate. Further, increasing concentrations of thrombin overcame the inhibitory effect of the patient's paraprotein. Thus, the patient's paraprotein appeared to possess antithrombin activity.

Topics: Aged; Antithrombins; Blood Protein Electrophoresis; Fibrin; Humans; Male; Paraproteinemias

Bleeding is a common complication in patients suffering from multiple myeloma. In some cases a defect in fibrin formation has been suggested as one possible cause of haemorrhagic tendency. As shown in this investigation the defect in fibrin formation, ascertained using PAGE, is due to a lack of alpha-chain polymerization of fibrin monomers in 5/11 patients with IgG myeloma and in 2/5 patients with IgM paraproteinaemia. No disturbed fibrin polymerization could be observed in IgA myeloma (n = 6). Factor XIII concentrations of subunit A and to a lesser extent of subunit S (Laurell technique) were highly elevated in all cases with regular fibrin formation. comparable values were obtained by measuring the transamidase activity of factor XIII by incorporation of 14C-labelled purtrescin into casein. Levels up to 600% of normal could be recorded. In contrast, all patients with a lack of alpha-chain polymerization had a factor XIII activity within the normal range. Addition of factor XIII concentrate to plasma from patients with defective fibrin formation led in 5/8 cases to a partial cross-linking of alpha-monomers. we conclude that in some cases paraproteins can inhibit the factor XIII and prevent its action on fibrin.

Topics: Blood Coagulation Tests; Electrophoresis, Polyacrylamide Gel; Factor XIII; Fibrin; Hemorrhage; Humans; Immunodiffusion; Immunoglobulins; Multiple Myeloma; Paraproteinemias

Topics: Fibrin; Hemorrhagic Disorders; Humans; Paraproteinemias; Polymers