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A 59-year-old male patient with Graves’ disease and severe hereditary Fletcher factor deficiency is described. PKK clotting activity as well as the activity by a chromogenic substrate method (Chromozym PK®) was less then 0.01 U/ml. In contrast to functional tests, the immunological assay (Laurell method) showed a PKK antigen concentration of 0.25 U/ml, indicating the presence of an abnormal nonfunctional PKK molecule (CRM+ variant). An inhibitor was excluded since the patient plasma did not inactivate partially purified PKK. Investigation of 11 family members revealed a reduction of the PKK clotting activity in 9 relatives of the patient. Since Graves’ disease is considered an autoimmune disease, our case represents an example of an association of a severe hereditary deficiency of a contact factor and an autoimmune disease.

12 plasmaphereses were carried out in 5 patients with antibodies to F VIII (3 haemophilic antibodies; 2 spontaneous antibodies). Plasmapheresis led in all instances to a marked reduction of the antibody level and there was a good correlation between the amount of plasma exchanged and the decrease of the antibody level. About 40 ml of plasma/kg body weight have to be removed to reduce the antibody level to half. In patients with low titre antibody who need treatment for serious bleeding, plasmapheresis is a more rapid and less expensive procedure than neutralisation of the inhibitor by high doses of F VIII. In one haemophiliac repeated plasmaphereses and subsequent high dose F VIII treatment eliminated the antibody within a short time. Plasmapheresis should always be considered when patients with antibodies to F VIII have to be treated because of severe bleeding.