[Skin and mucous membrane hemorrhages: clinical assessment, study sequence and relative frequency of hereditary diseases of the hemostasis in a Chilean population].

BACKGROUND

Skin and mucous membrane hemorrhages are distinctive manifestations of hereditary diseases of primary hemostasis and, among them, the different types of von Willebrand disease and of platelet function disorders are the most prevalent.

AIM

To know the relative frequency of these disorders and to know the clinical features of patients with mucocutaneous hemorrhages.

PATIENTS AND METHODS

Five hundred eighty nine patients whose main symptom was the presence of mucocutaneous hemorrhages were studied. Bleeding time, platelet count, coagulant activity of factor VIII (FVIII:C), FvW: Ag and FvW: CoRis and ABO blood group were measured in all patients in a first stage. According to the results of these tests, further studies were decided.

RESULTS

In patients younger than 13 years old, male predominated and, in older patients, females consulted with higher frequency. There was a higher proportion of individuals with O blood type than in the normal population. Bleeding time was abnormal in 330 patients (56%). One hundred ten patients (19%) had won Willebrand disease and, among them, one third had a normal bleeding time. Isolated reduction of factor WII activity was found in 66 patients (11%, 51 males) and 32 of these had normal bleeding time. Eighty one patients (14%) were considered to have an hereditary platelet function defect. A precise diagnosis was not achieved in 332 patients (56%).

CONCLUSIONS

Among patients consulting for mucocutaneous hemorrhages, 19% had von Willebrand disease, 11 had an isolated reduction of factor VIII activity, 14% had platelet function defects and in 56%, a precise diagnosis was not reached.