Incidence of infection with hepatitis B virus in 56 patients with haemophilia A 1971-1979.

During each of the four-year periods 1971-1975 and 1975-1979, the annual incidence of hepatitis B infection has been assessed in 56 patients with haemophilia A by measuring plasma HBsAg, anti-HBs and anti-HBc levels. Infection rates of 7% and 9.5% per annum respectively were observed for each four-year period despite the screening of individual blood donations for HBsAg by techniques up to the sensitivity of reversed passive haemagglutination. The highest incidence of seroconversion was amongst severe haemophiliacs many of whom had received treatment predominantly with cryoprecipitate. Of the 16 patients in whom serological evidence of hepatitis B infection was obtained only one had an accompanying clinical episode of hepatitis. We conclude that haemophiliacs are still at high risk of infection by hepatitis B virus despite the screening of individual blood donors for HBsAg.

Liver function in Edinburgh haemophiliacs: a five-year follow-up.

Liver function was assessed in 38 Edinburgh haemophiliacs. Results before the introduction of NHS intermediate factor VIII concentrate from 1974 onwards were compared with values in 1979. Measurements of serum bile salts in 16 patients as well as conventional liver function tests gave useful evidence of deranged liver function. Deterioration over the five-year follow-up period was seen only in patients on home treatment using large amounts of factor VIII concentrate, and there was no association between cryoprecipitate usage and derangement of liver function.

Cerebral vein thrombosis and the contraceptive pill in paroxysmal nocturnal haemoglobinuria.

A young woman who developed a cerebral venous thrombosis shortly after commencement of an oral contraceptive preparation was subsequently found to have paroxysmal nocturnal haemoglobinuria. The aetiology of thrombotic complications in this condition is discussed, with particular reference to the additional risk factor of the contraceptive pill in this case.

PIP: A case of a young women with paroxysmal nocturnal hemoglobin (PNH) who developed thrombosis of the cerebral veins after beginning a regimen of oral contraceptives is presented. She was 24 years old and presented with a 3-week history of frontal headache, neck stiffness, paraesthesiae of both arms, and weakness of the left leg. She had begun use of Microgynon 2 months before presentation, but had discontinued use when symptoms began. Hematological studies showed a shortened partial thromboplastin time, high fibrinogen and factor VIII levels, and prlonged euglobulin clot lysis time. Though this patient had a history of coagulation difficulties, it was not until after taking the estrogen-containing contraceptive preparation that PNH developed. The mechanism of thrombosis may be related to the liberation of thromboplastic material from hemolysed erythrocytes and to interaction between complement-sensitive platelets and complement components in plasma. It is suggested that the estrogen augmented the previously existing thrombotic condition in this patient, and that administration of estrogen-containing preparations should not occur in women with thrombotic disorders.

Köln haemoglobinopathy in a second Scottish family.

A second Scottish family with Haemoglobin Köln is described. Clinical and laboratory data on the two affected members are presented. The disorder appears to arise from a fresh mutation in the propositus.

Minimum effective dose of intermediate factor-VIII concentrate in haemophiliacs on home therapy.

The minimum effective dose of intermediate factor-VIII concentrate required for the successful home treatment of spontaneous joint bleeds in six haemophiliacs has been investigated in a randomised trial. 207 episodes of bleeding were included. 5 . 7 units/kg controlled 85% of bleeds, but with a dose of 3 .0 units/kg the risk of failure doubled. This lower dose is inadequate for treatment of bleeds other than those regarded subjectively by the patient as mild. The implications of these findings in terms of availability and usage of factor VIII in this centre are discussed.