The causes of death in a group of HIV-seropositive patients suffering
from congenital clotting disorders (cCD) were studied. During the past
6 years, we have followed 19 patients with cCD and HIV infection. Eig
ht patients fulfilled revised CDC criteria for AIDS, 6 subjects reache
d stage III of CDC, and 5 remained asymptomatic (CDC stage II). All pa
tients who developed AIDS died. In 5 patients, the terminal cause of d
eath was a severe haemorrhage (hematemesis, melena or haemoptysis) aft
er gastrointestinal or lung opportunistic infections. Two other patien
ts died as a consequence of disseminated infections but without signif
icant bleeding. Only one subject died due to neoplastic disease. In th
e first stages of CDC (II and III), no increase in bleeding symptoms w
ere seen in cCD HIV infected patients. The risk of haemorrhages is, ho
wever, increased in AIDS patients. Adequate replacement therapy should
be started early whenever severe opportunistic infections appear.