11/933. CO 2 laser surgery in hemophilia treatment. The use of CO 2 laser surgery between 1985 and 1991 in south africa and portugal for treatment of disorders in patients with mild to moderate cases of hemophilia A is discussed. Six cases of oral procedures and excision of skin tumors performed during this period are reported. In most of the cases of mild hemophilia no pre- or postoperative infusion of factor viii or desmopressin (DDAVP) was required. In some cases of moderate hemophilia, patients were infused with desmopressin (0.3 mug/kg body weight) and were treated postoperatively with the use of nasal desmopressin spray (150 mug to each nostril for four weeks following surgery). factor viii levels were measured before surgery. Follow up of four weeks was uneventful. The mean average power of the CO 2 laser was 20 W continuous and the pulse duration was 0.1 s for ablational procedures. For dermatologic procedures, a flexible plastic CO 2 laser hollow fiber was used (Flexilase, Sharplan, Allandale, NJ). We concluded that CO 2 laser surgery for hemophiliacs has a confirmed place in modern laser technology provided the standard precautions are taken and facilities are available. ( info) |
12/933. 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. ( info) |
13/933. CD4 depletion in hiv-infected haemophilia patients is associated with rapid clearance of immune complex-coated CD4 lymphocytes. The predominant immunological finding in hiv haemophilia patients is a decrease of CD4 lymphocytes during progression of the disease. Depletion of CD4 lymphocytes is paralleled by an increase in the proportion of immune complex-coated CD4 cells. We examined the hypothesis that the formation of immune complexes on CD4 lymphocytes is followed by rapid clearance of immune complex-coated CD4 lymphocytes from the circulation. In this study, the relationship of relative to absolute numbers of immune complex-loaded CD4 blood lymphocytes and their association with viral load were studied. Two measurements of relative and absolute numbers of gp120-, IgG- and/or IgM-loaded CD4 lymphocytes were analysed in hiv and hiv- haemophilia patients, with a median interval of approx. 3 years. Immune complexes on CD4 lymphocytes were determined using double-fluorescence flow cytometry and whole blood samples. viral load was assessed using NASBA and Nuclisens kits. Whereas the proportion of immune complex-coated CD4 lymphocytes increased with progression of the disease, absolute numbers of immune complex-coated CD4 lymphocytes in the blood were consistently low. Relative increases of immune complex-coated CD4 blood lymphocytes were significantly associated with decreases of absolute numbers of circulating CD4 lymphocytes. The gp120 load on CD4 blood lymphocytes increased in parallel with the viral load in the blood. These results indicate that immune complex-coated CD4 lymphocytes are rapidly cleared from the circulation, suggesting that CD4 reactive autoantibodies and immune complexes are relevant factors in the pathogenesis of AIDS. Relative increases of immune complex-positive cells seem to be a consequence of both an increasing retroviral activity as well as a stronger loading with immune complexes of the reduced number of CD4 cells remaining during the process of CD4 depletion. The two mechanisms seem to enhance each other and contribute to the progressive CD4 decrease during the course of the disease. ( info) |
14/933. 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. ( info) |
15/933. Acute renal failure complicating high-dose intravenous immunoglobulin therapy for acquired haemophilia. Acquired haemophilia is a rare disorder requiring therapy to control bleeding and to suppress the inhibitory antibody. High-dose intravenous immunoglobulin is commonly used as part of immunosuppressive regimens for this condition. We describe the case of an elderly patient who developed acute oliguric renal failure as a result of intravenous immunoglobulin therapy. All patients receiving such treatment should have renal function carefully monitored both during and after the infusion. ( info) |
16/933. 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. ( info) |
17/933. Does avascular necrosis cause collapse of the dome of the talus in severe haemophilia? Arthropathy of the ankle joint is commonly observed in people with severe haemophilia. A review of the radiological appearance of ankle arthropathy provides evidence that the changes in the talar bone of the ankle joint are probably due to avascular necrosis. This may arise due to impairment to the arterial supply, as it enters the talar neck, secondary to the increased pressure at the time of haemarthroses. ( info) |
18/933. 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. ( info) |
19/933. Management of liver failure in a haemophilic patient co-infected with human immunodeficiency and hepatitis c viruses. We present a case of liver failure in a haemophilic patient coinfected with transfusion acquired human immunodeficiency (hiv) and hepatitis c (HCV) viruses. The case illustrates the interaction of multiple viruses with accelerated progression to end stage liver disease and ultimately death. We report the impact on the patient management of two liver biopsies, which diagnosed an initial drug induced hepatitis and subsequently an atypical HCV related hepatitis. ( info) |
20/933. Characteristics of septic arthritis in human immunodeficiency virus-infected haemophiliacs versus other risk groups. The cases are presented of four haemophiliacs infected with human immunodeficiency virus (hiv) and with septic arthritis among the 340 patients followed at our centre. The data of these cases and 39 additional hiv-infected haemophiliacs with septic arthritis, identified in a literature search, are reviewed. The spectrum of bacterial pathogens is limited and somewhat different from that in other risk groups. The localization is exclusively to joints affected by haemophilic arthropathy. The laboratory picture is characterized by the absence of peripheral leucocytosis, varying CD4-helper cell counts, a high erythrocyte sedimentation rate and fever. The clinical picture mimics that of haemarthrosis, often causing a delay in diagnosis. Treatment with systemic antibiotics is often sufficient, obviating the need for arthrotomy and open drainage. prognosis related to the joint function is relatively good, but poor when related to the medium- to long-term survival of the patient. ( info) |