[Desmopressin in hemophilia A and Von Willebrand's disease]. / Desmopressine bij hemofilie A en de ziekte van Von Willebrand.

The treatment with desmopressin prior to surgery of patients with mild haemophilia A (HA) and Von Willebrand's disease (VWD) was retrospectively evaluated in a general hospital, from 1978 until 1987. From a group of 87 treated patients, 40 patients are reported (21 VWD, 19 HA) of which plasma factor VIII (FVIII) and Von Willebrand factor (VWF) concentrations were determined before, and twice after desmopressin treatment. Desmopressin was administered intravenously at a dose of 0.4 micrograms/kg body weight. Tranexamic acid was used only when surgery in the mouth cavity was performed, at a dose of 1 gram three times a day. Side effects were seen only in 5 patients (3 VWD, 2 HA). No significant difference between both groups was seen in bleeding tendency, transfusion necessity and side effects (chi 2 test). In both groups, FVIII and VWF concentrations increased significantly after 20 and 60 minutes following DDAVP administration (paired t-test). After 360 minutes, the FVIII concentration increased significantly in both groups, however, only in the VWD patients did VWF increase significantly. In neither group did initial FVIII concentrations correlate with the increase in FVIII (linear regression analysis). One female patient reacted differently to DDAVP, with a decrease in FVIII and VWF values. Desmopressin is a safe and effective agent in the management and prophylaxis of bleeding tendency in patients with mild HA and mild VWD.

A patient with a spontaneous factor VIII:C autoantibody: successful treatment with cyclosporine.

A 65-year-old man was admitted to the hospital because of extensive spontaneous ecchymoses of the trunk and huge hematomas of the arms and legs. He had no personal or family history of a hemorrhagic diathesis. Coagulation studies revealed a prolonged APTT, no detectable factor VIII:C activity, and a high titer of anti-factor VIII:C antibodies. A diagnosis of acquired hemophilia was made. No underlying disorder could be found. The inhibitor was an IgG antibody. Long-term management of bleeding including immunodepletion by plasma exchange and immunosuppression by corticosteroids and cytotoxic drugs alone and in combination had no effect on the bleeding tendency and coagulation data. The administration of Cs (10 mg/kg/d) in combination with prednisone induced a remission. After a stormy course and a 5-month stay in the hospital the patient could be discharged. A relapse occurred after the Cs and prednisone dosages were reduced. Increasing the Cs dosage induced a remission again.