Internal Medicine

0521779407-09 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:12

676 Hemophilia A and B

Contraindications

■Occasional patients with severe hemophilia B develop anaphylactic

type reactions to FIX infusions.

■For patients with inhibitors, factor infusion is not harmful but is

expensive and non-efficacious and should be avoided as a strategy

for controlling bleeding except in cases of low titer inhibitor (vide

infra).

follow-up

■For uncomplicated joint bleeds, range of motion should improve

over course of few days.

■For minor bleeds, check factor levels only if clinical response is sub-

therapeutic or if questions arise about dosing. For major or life-

threatening bleeds, important to follow factor levels during treat-

ment.

complications and prognosis

■Most common causes of mortality in severe hemophilia in US are

HIV-related complications; hepatitis-related complications; bleed-

ing complications.

■Current life expectancy for severe hemophiliac not infected with HIV

or hepatitis is 63 years of age. Most individuals <22 years of age are

free of HIV and hepatitis, many >22 yrs have been exposed.

■HIV disease: >80% of those with severe hemophilia A infected in late

1970s through early 1980s from plasma-derived concentrates.% was

slightly less for those with hemophilia B.

■Many now on standard HAART regimens. A few reports have sug-

gested greater frequency of bleeding episodes on protease inhibitors;

treaters should be alert for this problem.

■Hepatitis C: >90% of those with severe hemophilia infected during

1970s and 80s. Natural history of disease in hemophilia population

similar to that seen in other affected groups. Co-infection with HIV

may accelerate progression of liver disease.

■Inhibitory antibody formation is now major complication of treat-

ment – occurs in 25% of hemophilia A, 3% of hemophilia B.

➣Inhibitors prevent successful treatment with clotting factor con-

centrates.

➣Diagnosis: clinically patient fails to respond to infused concen-

trate; laboratory: 1:1 mix does not correct, inhibitor is quantitated

using Bethesda assay.

➣Inhibitors can be transient or long-lasting, and low or high titer.