[Infective morbidity of replacement therapy in congenital coagulation deficiencies and its effects on demand of coagulation factors].

BACKGROUND

Analysis of the infective morbidity and mortality secondary to replacement hemotherapy in the population with congenital coagulation deficiencies (CCD) and their consequences on the demand for coagulation factors.

METHODS

The 46 patients with CCD diagnosed in the autonomous community (AC) of the Balearic Islands (32 with hemophilia A, 6 with hemophilia B, 4 with von Willebrand's disease 2 with factor VII, 1 with factor X, and 1 with factor XII deficiencies) were investigated for infective morbidity and use of blood products from 1982 to 1987.

RESULTS

97% of the patients had some hepatitis marker, 77% had antibodies against human immunodeficiency virus (HIV) and 17% fulfilled the criteria for the acquired immunodeficiency syndrome (AIDS). There were 7 deaths (15%). The morbidity and mortality increased with age and use of blood products. There was a 46% reduction in factor VIII use between 1982 and 1986, from a mean yearly consumption per hemophiliac patient of 33444 international units (IU) to 18080 IU.

CONCLUSIONS

The study results show a high prevalence of hepatitis and HIV, an important reduction in the demand of manufactured coagulation factors and a 15% reduction in the population with CCD during the study years.