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2013, Number 6

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Rev Med Inst Mex Seguro Soc 2013; 51 (6)

Hemophilia

Amador-Medina LF, Vargas-Ruiza ÁG

Language: Spanish
References: 32
Page: 638-643
PDF size: 63.28 Kb.


ABSTRACT

Hemophilia is a genetic disease in which the clinical manifestation is mainly the presence of hemorrhage. There are two known types of hemophilia: hemophilia A and B, which have a defi ciency of factor VIII or IX clotting, respectively. The intensity of bleeding in hemophilia depends on the plasma levels of factor VIII or IX and has traditionally been classifi ed as mild (≤ 5 % activity), moderate (1-5 % activity) and severe (‹ 1 % activity). In laboratory tests, it can be found isolated prolongation of activated partial thromboplastin time (aPTT), but it is necessary to determine the plasma levels of factor VIII or IX to establish the diagnosis of hemophilia A or B. The treating of this disease involves replacing exogenous factor VIII or IX concentrates. Gene therapy could be an option in the future to achieve the cure of the disease. Complications of hemophilia are the risk of transfusion-associated infections, pseudo tumor hemophilic, hemophilic arthropathy and the presence of serum inhibitors.


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