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2011, Number 4

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Rev Hematol Mex 2011; 12 (4)

Prophylaxis in haemophilia: experience of a tertiary hospital

García-Candel F, Cabañas-Perianes V, Moreno-Moreno M, Majado J, Salido-Fierrez E, Moraleda JM

Language: Spanish
References: 33
Page: 249-256
PDF size: 108.49 Kb.


ABSTRACT

Background: Hemophilia A and B are hereditary coagulopathies caused by deficiency of factor VIII or factor IX. Hemophilic arthropathy is the major cause of morbidity in patients with severe hemophilia. It has recently been suggested that prophylactic administration of human concentrates of the deficient factors is an effective method to prevent hemophilic arthropathy and its sequelae.
Patients and Methods: We retrospectively analyzed clinical and laboratory data of five patients with severe hemophilia A, two with antifactor VIII inhibitor, and two patients with severe hemophilia B, that were included in a prophylaxis program during the years 2000-2009 in Murcia, Spain. The genetic studies were performed by standard techniques of molecular biology. From the clinical data we performed a descriptive analysis of the dosage and schedule of the coagulation factor concentrate used, and the frequency and type of bleeding episodes during prophylaxis.
Results: In patients who began the program of secondary prophylaxis there was a trend to a lower occurrence of overall bleeding episodes. Patients with severe hemophilia A in primary prophylaxis had fewer bleeding events than those on secondary prophylaxis. In patients with a history of anti-factor VIII inhibitor, the bleeding episodes decreased when the prophylaxis with a by-pass coagulation agent was initiated before starting the immune tolerance.
Conclusions: The results of our small series suggest that prophylaxis with recombinant coagulation factors is a feasible, safe and effective procedure to prevent hemarthrosis even in patients with repetitive episodes.


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