Haemophilia : the official journal of the World Federation of Hemophilia
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Review Case Reports
Clinical manifestations and management of labor and delivery in women with factor IX deficiency.
Haemophilia is uncommon in females and little is known about the clinical manifestations and postpartum management of women with this disorder. Clinical characteristics of postpartum bleeding were evaluated in women with factor IX deficiency (FIX:C < 0.20 U mL(-1)), including two with haemophilia B and three carriers of haemophilia B, undergoing labour and delivery. Data were collected prospectively during routine outpatient comprehensive haemophilia care at the haemophilia Center of Western Pennsylvania and during inpatient management. ⋯ Caesarean, nor the FIX level (P = 0.371; FIX > 0.05 U mL(-1) vs. < or =0.05 U mL(-1)). Compared with females with von Willebrand disease or FXI deficiency, females with FIX deficiency were more likely to experience postpartum bleeding (P = 0.008) and anaemia (P = 0.045); and they were less likely to experience menorrhagia (P = 0.065), but the latter did not reach significance. Postpartum bleeding is common in women with haemophilia B or carriers of haemophilia B, and treatment with factor replacement for at least 4 days of postpartum may prevent bleeding following delivery in such women.
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The rare coagulation disorders are heritable abnormalities of haemostasis that may present significant difficulties in diagnosis and management. This review summarizes the current literature for disorders of fibrinogen, and deficiencies of prothrombin, factor V, FV + VIII, FVII, FX, the combined vitamin K-dependent factors, FXI and FXIII. Based on both collective clinical experience and the literature, guidelines for management of bleeding complications are suggested with specific advice for surgery, spontaneous bleeding, management of pregnancy and the neonate. We have chosen to include a section on Ehlers-Danlos Syndrome because haematologists may be consulted about bleeding manifestations in such patients.
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The development of neutralizing antibodies to factor VIII or IX therapeutic concentrates remains the most serious and challenging complication in the management of patients with haemophilia A and B. FEIBA, Anti-Inhibitor Coagulant Complex, is an activated prothrombin complex concentrate that has been used to treat patients with such complications for almost 30 years. The mechanism of action of FEIBA has been proposed to involve simultaneous FVIII/FIX inhibitor bypassing action in the common, intrinsic and extrinsic coagulation pathways. ⋯ The incidence of thrombotic adverse events recorded in the Baxter pharmacovigilance database for the 10-year postmarket period (1990-99) was approximately 4 : 100,000 infusions of FEIBA. Almost all documented thrombotic events with FEIBA occurred with doses that exceeded dosing recommendations, and known risk factors for cardiovascular disease were evident in more than 80% of the patients involved. Overall, clinical data have shown FEIBA to be safe and well-tolerated for use in a wide variety of clinical settings, including treatment of bleeding episodes, management of surgical procedures, home therapy, long-term prophylaxis, and prophylaxis during immune tolerance induction, when used according to dosing guidelines.
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Endometrial ablation has recently gained popularity as a treatment of menorrhagia in the general population. In the von Willebrand disease (VWD) patient, intuitively, it would appear that the failure rate would be higher because of the underlying hypocoaguability increasing the likelihood for re-bleeding. In a consecutive series of seven patients, we assessed the efficacy and safety of endometrial ablation in VWD-related menorrhagia. ⋯ Endometrial ablation appears to be a safe procedure that improves the QOL in patients with VWD-related menorrhagia. However, its long-term efficacy appears to be lower in VWD patients when compared with women with menorrhagia without VWD.