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In a group of six patients suffering from various diseases accompanied by splenomegaly, low levels of factor V and fibrinogen have been observed apart from thrombo-cytopenia. No circulating fibrin degradation products (FDP) and fibrin monomers were present and anticoagulants did not correct the coagulation disorder. However, other findings, such as the presence of fibrin deposits in the enlarged spleen, and correction of the hemostatic defect after splenectomy strongly suggest local consumption of platelets and clotting factors in the enlarged spleen. The data obtained show that a decreased concentration of clotting factors in patients with splenomegaly can be caused by local consumption alone. The absence of an elevated FDP level distinguishes our findings from those found in consumption coagulopathy accompanying diffuse intravascular clotting (DIC).

The effect of high-dose intravenous gammaglobulin (IVG) therapy with a CLB preparation was studied in 42 patients: 8 patients had acute and 26 patients had chronic idiopathic thrombocytopenic purpura (ITP); 5 patients had thrombocytopenia accompanied by various diseases such as systemic lupus erythematosus, auto-immune haemolytic anaemia and neutropenia; 3 patients had hypoplastic anaemia and 1 patient had neutropenia and rheumatoid arthritis. After treatment, a rise in platelet count occurred in about 75% of the patients with ITP, although there was no sustained response in any of the patients. There was no correlation between the strength of platelet antibodies as detected by the direct immunofluorescence test before infusion and the pattern of response to the infusion. In most cases of ITP, no immune complexes, as measured by Clq-binding assay, were observed. Furthermore, we found no relationship between the amount of Clq-binding activity of patients' sera and the reaction pattern after infusion of IVG. Splenectomy of the patient had no influence on the outcome of IVG therapy.