Hemophilia complications

List the complications from hemophilia bleeding episodes. 

The main goal of hemophilia treatment is to prevent bleeding episodes and their long-term complications. Clinicians should evaluate patients every 6 to 12 months for the following ... 

A serious complication of HIV infection is HIV-associated thrombocytopenia, This results from immune-mediated platelet destruction and decreased or defective platelet production due to infection of megakaryocytes with HIV-1 (211). HIV-related thrombocytopenia may be associated with an accelerated progression to AIDS and decreased survival rates. Hence management of thrombocytopenia in AIDS patient is crucial to prevent severe complications, Severe bleeding complications in HIV-infected hemophilia patients treated with protease inhibitors... 

Some hemophilia A and B patients develop antibodies against FVIII and FIX, respectively. This complicates the direct administration of these proteins. An alternative in these cases is treatment with active factor VII (FVIIa), which complexes with factor III in the presence of phospholipids and Ca2+, activating factor X, which, in normal patients, is activated by active factors VIII and IX. The commercial name of recombinant FVIIa expressed in BHK cells and produced by Novo-Nordisk is NovoSeven (Table 16.1). 

Thrombophlebitis at the infusion site is a common complication of continuous infusion of various clotting factor concentrates and has been noted after infusion of factor Vila (9,10). Thrombophlebitis occurred in one of eight hemophiliacs with inhibitors who received continuous infusion of recombinant factor Vila to allow elective surgery (11). In 25 hemophilia patients with inhibitors, who received recombinant factor Vila for surgical procedures or spontaneous bleeding, there was one case of thrombophlebitis in 35 continuous infusion courses (12). In most instances, thrombophlebitis can be prevented by parallel infusion of saline or heparin. 

This may explain the effectiveness of treating patients with hemophilia who have inhibitors with high doses of factor VIII. Another approach involves the use of prothrombin complex concentrate to treat bleeding episodes in patients with factor VIII inhibitors (38) however, thromboembolic complications related to higher doses of prothrombin complex concentrate have been described, although these are relatively rare (39,40). Thrombotic events are extremely rare when highly purified factor IX is used. Activated prothrombin complex concentrate is also effective in patients with factor VIII inhibitors (41). Serious complications are rare, but disseminated intravascular coagulation has been reported (42). 

The administration of prothrombin complex concentrate to reverse anticoagulant treatment has been associated with thrombotic complications, such as disseminated intravascular coagulation (8). However, such complications have typically occurred in patients with liver failure or after repeated treatment, such as in patients with hemophilia B treated with prothrombin complex concentrate (9). 

Another major complication in the treatment of hemophilia A is the occurrence of inhibitors against FVIII (neutrahzing antibodies) in about 30% of patients, usually within the first 100 exposure days. Patients with severe hemophilia A (FVIII levels... 

A 8-month-old boy with hemophilia B was given plasma-derived factor IX concentrate and after 1 month developed recurrent anaphylactic reactions following infusions of factor IX, with shortness of breath, wheeze, and widespread urticaria [70 ]. High-dose factor IX immune tolerance therapy was successful in eradicating the inhibitor for 40 months. Two more attempts to achieve immune tolerance with high-dose factor IX were complicated by recurrent anaphylactic reactions. 

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