Cases reported "Hemophilia A"

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1/119. Treatment of congenital and acquired hemophilia patients by extracorporeal removal of antibodies to coagulation factors: a review of US clinical studies 1987-1990. Hemophilia Study Group.

    This paper reviews the use of extracorporeal immunoadsorption with immobilized staphylococcal protein a in attempts to lower the inhibitor titer in 22 patients with either congenital hemophilia or with acquired inhibitors. Eighty-five immunoadsorption procedures were performed at 13 locations in the united states between June, 1987 and February, 1990. In general, immunoadsorption was shown to efficiently remove IgG and, in eight congenital hemophilia patients, it also produced a clinically significant lowering of inhibitors allowing effective conventional factor replacement therapy. Three of thirteen congenital hemophilia patients treated received factor concentrate prior to immunoadsorption and were anamnestic at the time of treatment. Although they experienced substantial lowering of their inhibitor titers, it was not sufficient to allow effective factor replacement. The effectiveness of immunoadsorption therapy in the 9 patients with acquired inhibitors was more difficult to evaluate due to the wide variety of concomitant medications which were employed, although in several patients serious bleeding episodes were substantially improved (or halted) following immunoadsorption. Side effects associated with immunoadsorption were slight. These findings suggest that immunoadsorption can be a significant benefit to patients with inhibitors, particularly if it is instituted prior to factor administration.
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2/119. Joint replacement for a spontaneously ankylosed hip in a haemophilic patient.

    A cemented Charnley total hip prosthesis was implanted in a 48-year-old man with mild haemophilia (factor viii 4 IU dL-1) in his right spontaneously ankylosed hip. At the time of surgery he was anti-HCV positive, anti-hiv negative, and no circulating inhibitors were encountered. The indication for surgery was long-lasting intractable low back and ipsilateral knee pain. At 4-month follow-up, relief of pain was achieved as well as correction of limb-length discrepancy, with a good result according to the Mayo Clinic hip score. Doses of 50 IU kg-1 body weight of recombinant factor viii (Recombinate; Baxter, Glendale, california, USA) was used during the 2 weeks of admittance to the hospital. The dosage was adjusted according to the recoveries of factor viii, with an overall factor consumption of 68 000 IU. As far as we know this is the first case reported in the literature of a person with haemophilia in whom a spontaneous hip ankylosis has been satisfactorily converted in a total hip arthroplasty with a short-term follow-up. However, a much longer follow-up is still needed to ascertain the efficacy of this surgical procedure in haemophilia.
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3/119. Postoperative use of rFVIIa by continuous infusion in a haemophilic boy.

    Continuous infusion of coagulation factor concentrates has proved to be safe and effective. Because rFVIIa (NovoSeven is a very expensive product and very frequent doses are needed, continuous infusion is expected to be highly cost-effective. The postoperative use of continuous infusion of rFVIIa in a haemophilic boy with a high titre FVIII inhibitor is reported. He presented with a large right knee haemarthrosis and was treated with intermittent doses of rFVIIa. After a transient improvement the haemarthrosis became worse and an open evacuation of the joint had to be made under treatment with bolus injections of rFVIIa for 3 days (120 microg kg(-1) every 2 h). A previous pharmacokinetic evaluation in this patient had showed that FVIIa recovery and half-life were less than expected. Continuous infusion of rFVIIa (20 microg kg(-1) h(-1)), with added low molecular heparin to prevent local thrombophlebitis, was started on the fourth postoperative day and maintained unchanged for 7 days. Four additional single bolus injections were given for early joint mobilization. The intervals between replacements of the pump syringes were progressively increased from 6 to 12 h and then up to 24 h. FVIIa plasma levels during continuous infusion ranged between 6.3 and 10.4 IU mL(-1). Although FVIIa assays seemed to show good stability, we observed the formation of precipitates inside the syringes. The precipitates seemed to contain FVIIa. We concluded that FVIIa plasma levels of 6-10 IU mL(-1) were safe and effective to prevent postoperative haemorrhage in this patient. The addition of heparin to the rFVIIa concentrates, however, may cause precipitation and should be avoided. Individual pharmacokinetic evaluation may be useful to select the appropriate initial doses, especially in young patients.
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4/119. Hemophilic pseudotumor of the soft tissue of the hand: a case report.

    A 25-year-old, right hand dominant man with a history of hemophilia a, presented with an enlarging 2 x 3.5 cm mass on the dorsum of his dominant hand following minor trauma. factor viii activity level was 12 percent of normal. A diagnosis of hemophilic pseudotumor was made and the mass continued to enlarge despite medical management. Surgical intervention was carried out in order to prevent impending skin necrosis or rupture. The patient was treated by surgical excision of the lesion along with factor replacement therapy. The patient's symptoms resolved and he had no recurrence of the tumor.
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keywords = replacement therapy, replacement
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5/119. Nasopharyngeal angiofibroma in a patient with haemophilia A: a bleeding tumour in a bleeding-prone patient.

    Nasopharyngeal angiofibroma is a highly vascular tumour which occurs almost exclusively in adolescent males. Although it is histologically benign, it may cause serious clinical problems because of its tendency to bleed profusely during surgery. This paper presents the first case of nasopharyngeal angiofibroma in a patient with haemophilia A, another well-known disease of bleeding tendency. In this case the tumoural mass was surgically removed with effective factor viii replacement without any bleeding complication.
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6/119. Use of recombinant factor viia (NovoSeven) in a haemophilia A patient with inhibitor in kuwait.

    Development of inhibitors is a known complication in some haemophiliacs receiving coagulation factor replacement therapy. We report on the successful management of a young boy with haemophilia A with inhibitor using recombinant factor viia. We had failed to control bleeding in this patient following his circumcision, despite infusion with high doses of factor viii concentrate for 2 weeks. Recombinant factor viia is a useful 'factor viii bypassing agent' for the control of bleeding in patients with haemophilia A and B who develop inhibitors. We suggest that severely affected haemophiliacs should be absolved of ritual circumcision as a protective measure against what might become a life-threatening haemorrhage - especially in those with inhibitors.
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keywords = replacement therapy, replacement
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7/119. Manifestations of factor viii inhibitor in the head and neck.

    hemophilia a is the most severe of the inherited bleeding disorders. Otolaryngologists are frequently asked to assist in the care of a patient with this disorder who has intractable bleeding from sites in the head and neck. This disorder is transmitted in an X-linked recessive manner and results from factor viii deficiency. Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae in patients with hemophilia. A consequence of factor replacement therapy that occurs in as much as 16% of patients is the development of antibodies to the exogenous factor. These antibodies inactivate any supplemental factor viii, resulting in continued bleeding despite attempts at replacement therapy. This poses an extremely challenging clinical problem because other standard therapies have had limited usefulness. Activated prothrombin complex has been shown to be efficacious in the treatment of patients with inhibitors. This has led to the production of factor viii inhibitor bypassing activity (FEIBA), which contains activated forms of factors II, VII, IX, and X. The mechanism of action of this preparation remains unknown, but it seems to bypass the need for factor viii in the clotting cascade. factor viii inhibitor bypassing activity is not as effective as factor viii concentrates in patients with normally responsive hemophilia, and patients with low inhibitor levels may be given larger doses of factor viii. disseminated intravascular coagulation is most often mentioned as a potential complication of the administration of factor viii inhibitor bypassing activity, although it has been rarely reported. Otolaryngologists should be familiar with this clinical phenomenon and understand its implications. Two patients with bleeding diatheses were recently treated at our institution.
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keywords = replacement therapy, replacement
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8/119. Management of haemophilia in patients with high-titre inhibitors: focus on the evolution of activated prothrombin complex concentrate AUTOPLEX T.

    Numerous therapeutic strategies have been applied to the management of patients with inhibitors to factors VIII or IX. Different treatment approaches are analysed including prothrombin complex concentrates (PCCs), activated prothrombin complex concentrates (aPCCs), porcine factor viii concentrate, inhibitor neutralization, immune tolerance therapy, immunosuppressive regimens and recombinant factor viia. Clinical data are reported in the analysis of several treatments. PCCs and aPCCs have gained widespread acceptance as the standard first-line approach for patients with inhibitors. The aPCC AUTOPLEX T has achieved a high response rate with a low level of thrombotic events. Four case studies are presented in which AUTOPLEX T has been used successfully. Administration of platelet concentrate or, in elective surgery, waiting for inhibitor levels to decline are useful adjuncts to some treatments. The optimal treatment depends on the patient's inhibitor status--low responder (minimal or no increase in inhibitor levels upon administration of replacement clotting factor) or high responder (replacement clotting factor generates inhibitor production). A suggested algorithm for treating high-responder inhibitor patients is presented.
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ranking = 0.14094389279655
keywords = replacement
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9/119. plasma exchange for acquired hemophilia: a case report.

    A patient with acquired hemophilia complicated with chronic renal failure and lung tuberculosis was successfully treated by consecutive plasma exchange (PE). A 71-year-old man with serious bleeding tendency showed low coagulation factor levels and a high titer of factor viii (FVIII) inhibitor, and he was diagnosed with acquired hemophilia. Because of the complication of active lung tuberculosis, instead of immunosuppressive therapy, he undertook a series of PE with fresh frozen plasma replacement for 3 months. After the start of PE, his bleeding symptoms and activated partial thromboplastin time (APTT) improved gradually according to the decrease in FVIII inhibitor levels. These results suggest that PE is an effective therapeutic tool for refractory acquired hemophilia.
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keywords = replacement
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10/119. Thrombosis in a duplicated superficial femoral vein in a patient with haemophilia A.

    venous thrombosis is a very rare occurrence in patients with haemophilia A. We report the case of a haemophiliac in whom initially a calf haematoma was suspected, but neither this nor deep venous thrombosis (DVT) could be confirmed on ultrasound scanning. Subsequently, a high segment venous thrombosis was diagnosed by venography in a portion of a duplicated superficial femoral vein. Treatment with factor viii (FVIII) and low molecular weight heparin led to a successful resolution. The only other case we have been able to find in the literature occurred during FVIII replacement therapy, which was not the situation with our patient.
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keywords = replacement therapy, replacement
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