1/129. 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.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
2/129. 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.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
3/129. Acquired factor viii inhibitor in a non-hemophilic patient with chronic hepatitis c viral infection.Production of coagulation factor viii inhibitor is rarely encountered in non-hemophilic patients. A 63-year-old Japanese male suffered from severe bleeding tendency caused by this inhibitor. Although he did not have malignancy or collagen disease, he had chronic hepatitis c virus (HCV) infection. Although HCV is known to induce production of various autoimmune antibodies, this may be the first report of a case with both acquired factor viii inhibitor and HCV infection.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
4/129. The non-surgical treatments of hepatocellular carcinomas in a patient with severe hemophilia a.We recently treated a patient with multiple hepatocellular carcinomas (HCC) who had severe hemophilia a and thrombocytopenia secondary to cirrhosis. He was not a candidate for surgery because of his liver failure. Transcatheter arterial chemoembolization and percutaneous ethanol injection therapy were successfully performed without complications by maintaining factor viii levels above 40%. We believe that the non-surgical treatment of HCC can be performed safely in patients with coagulation disorders, by the appropriate administration of coagulation factors, despite thrombocytopenia and/or elongation of the prothrombin time from cirrhosis.- - - - - - - - - - ranking = 4.4562329112115keywords = coagulation disorder, coagulation (Clic here for more details about this article) |
5/129. 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.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
6/129. Long-lasting remission and successful treatment of acquired factor viii inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus.Acquired deficiency of clotting factor viii (FVIII) is a rare bleeding diathesis seldom encountered in systemic lupus erythematosus (SLE). Reduction of FVIII activity by autoantibodies can cause potentially life-threatening situations. Herein, an SLE patient with a positive lupus anticoagulant (LAC) test who abruptly developed metrorrhagia 4 yr after diagnosis is reported. Coagulation tests revealed FVIII activity reduced to 3% and a prolonged aPTT. FVIII inhibitor(s) were found to be as high as 3.0 Bethesda Units. plasmapheresis, immunoglobulins, prednisolone and FVIII plasma concentrates induced the cessation of metrorrhagia, but the clotting tests were barely improved. One month later, extensive ecchymosis appeared and worsened, despite re-administration of the previous therapy. pulse cyclophosphamide followed by oral administration was then started with normalization of coagulation parameters and long-lasting disease remission.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
7/129. Surgery-associated factor viii inhibitors in patients without hemophilia.The acquisition of antibodies to factor viii (ie, factor viii protein) by patients without hemophilia is associated with conditions such as pregnancy, lymphoma, and autoimmune disorders. We present three patients who acquired factor viii antibodies in the postoperative setting. Preoperatively, none gave a history of bleeding even with past surgeries and all had normal coagulation tests. Within days of surgery (bowel resection, cholecystectomy, coronary bypass), a bleeding diathesis emerged with prolonged partial thromboplastin time, decreased factor viii levels, and demonstrable factor viii antibodies. All patients required multiple blood transfusions and urgent reexploration for hemostatic control. All were treated with high dose human factor viii infusions, corticosteroids, and various forms of immunosuppression. We wish to raise awareness of surgery as a precipitating cause of acquired hemophilia, which is something to be considered with unusual postoperative bleeding. This syndrome is remarkable for its abrupt onset within days of surgery, dramatic bleeding, subsequent persistence, but potential reversal by immunosuppression.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
8/129. 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.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
9/129. Major surgery for a gastric cancer in a haemophilic with high inhibitor titre successfully performed by the use of recombinant FVIIa.A total gastrectomy with omentectomy and resection of the distal oesophagus in a 69-year-old haemophilia A patient with high inhibitor of 128 Bethesda units is described. Surgery was successfully performed after infusion of 112 microg kg-1 bw of recombinant FVIIa. Ninety-two microg kg-1 were given thereafter at time intervals of 2 h until 12 h, then every 3 h until 24 h, and every 4 h until 48 h after surgery. Doses were gradually reduced in the following days and finally discontinued on day 28 after surgery. The complete treatment schedule required the administration of a total of 708 mg of recombinant FVIIa. Using this approach, we observed normal haemostasis, and there were no signs of excessive postoperative bleeding or wound haematoma. No clinical side-effects or evidence of systemic activation of coagulation occurred during the treatment. As judged from the clinical course of this major surgery, recombinant FVIIa appears to be highly efficacious and safe and should be used as first line treatment in high titre inhibitor patients with cross-reactivity to porcine factor viii, undergoing surgery.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
10/129. 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.- - - - - - - - - - ranking = 0.25keywords = coagulation (Clic here for more details about this article) |
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