HIV-disease progression in terms of the decline in CD4(+) cell count,
the development of AIDS-related symptoms and death was studied in 100
Swedish HIV-positive haemophiliacs and correlated to age and haemophil
ia treatment. On average 15 years after seroconversion, 66% of the pat
ients had CD4(+) cell counts of < 200 x 10(6) L-1, 48% had developed A
IDS and 56% had died. Age was found to correlate to all three endpoint
s, also after adjustment for age, annual closing factor concentrate (C
FC) consumption and HIV-related therapy, i.e. pneumocystis prophylaxis
and antiretroviral drugs (P < 0.05). Total annual CFC consumption sho
wed no significant relationship to the decline in CD4(+) cell counts b
ut was inversely correlated to both the development of AIDS-related sy
mptoms (P = 0.033) and mortality (P = 0.014). Prophylactic treatment w
as not associated with significantly better survival than on-demand tr
eatment after adjustment for age, CFC consumption and HIV-therapy. The
use of monoclonal-antibody-purified CFCs was not found to stabilize t
he decline in CD4(+) cell counts. However, the use of these CFCs was i
nversely correlated both to the development of AIDS-related symptoms a
nd to mortality (P = 0.042 and 0.027, respectively). A similar trend w
as associated with the use of low-and intermediate-purity CFCs. As com
pared with the severe haemophilia A subgroup, the moderate haemophilia
A patients showed a trend toward slower disease progression, possibly
attributable to a lower incidence of haemarthrosis and arthropathy am
ong the latter. We conclude that replacement therapy in HIV-infected h
aemophiliacs is important also for HIV-disease progression, whereas th
e purity of the CFCs and the regimen used are of minor importance.