Cases reported "Hemophilia A"

Filter by keywords:



Filtering documents. Please wait...

1/15. Experience with continuous infusion of recombinant activated factor VII in the asia-Pacific region.

    There is increasing interest in continuous infusion of recombinant activated factor VII (rFVIIa) as a convenient and safe alternative to intermittent bolus therapy. In the Australian patients reported in this paper, cost savings of up to 25% in the first 12 h of treatment with continuous infusion of rFVIIa have been achieved safely, suggesting that substantial overall savings are possible. However, in the Thai patient reported, a dose reduction of 35% in the first 12 h was associated with poor haemostatic control, suggesting that a dose reduction of >25% may be inadvisable. The indications for treatment in the five Australian patients were: retroperitoneal haemorrhage (n = 3); right forearm compartment syndrome (n = 1); wrist haemarthrosis and median nerve compression (n = 2); sublingual haematoma (n = 1); and cerebral (mid-brain) haemorrhage (n = 1). Treatment was effective in four out of five patients (six bleeding episodes) and there was one treatment failure where treatment had been substantially delayed. The Thai patient was treated as part of a prospective, uncontrolled, observational study of 34 bleeding episodes in 22 patients in the asia-Pacific region. Treatment was judged ineffective after 24 h, but full haemostatic control was subsequently achieved with intermittent rFVIIa therapy.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

2/15. Acute left anterior descending artery occlusion in a hemophiliac A patient during recombinant factor viii infusion: treatment with coronary angioplasty.

    Acute myocardial infarction in association with the replacement of recombinant factor viii in hemophiliacs has not been documented. We describe the use of PTCA in a hemophiliac A patient who developed acute myocardial infarction during factor viii replacement. Because surgery in hemophiliac A patients remains hazardous, PTCA seems to be an attractive alternative.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

3/15. Hemophilic pseudotumor of the ulna treated with low dose radiation therapy: a case report.

    We report a case of hemophilic pseudotumor in the ulna of a 6-year-old boy treated with radiation therapy. A total dose of 900 cGy in 6 fractions was given in 6 consecutive days. Progression of cystic changes was halted within a month. New bone formation and trabeculation were found on the 4th month. Complete healing of the lesion and bony replacement were found on the 12th month. The patient was followed up to 72 months and there was no evidence of recurrence and no bone growth disturbance. radiation therapy can be an effective alternative modality in treating hemophilic pseudotumor.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

4/15. Immunoadsorption may provide a cost-effective approach to management of patients with inhibitors to FVIII.

    BACKGROUND: Immunoadsorption of plasma with staphylococcal protein a removes immunoglobulins and immune complexes; hence, it should effectively remove inhibitors to FVIII in acquired or congenital hemophilia. The procedure may be cost effective, given the expense of therapies used to treat patients with inhibitors, particularly in an acute setting. STUDY DESIGN AND methods: Three patients with inhibitors to FVIII were treated with the Excorim Immunosorba system (two columns used in tandem). Costs for immunoadsorption and for other therapeutic products administered to the patients were calculated. RESULTS: Two patients had acquired hemophilia and severe bleeding associated with low levels of circulating FVIII and high levels of inhibitors to FVIII. They failed to achieve a satisfactory response to management with immunosuppression, pFVIII, recombinant FVIIa or IVIG but responded rapidly, with long-term benefit, to immunoadsorption therapy. The third patient had congenital hemophilia and immunoadsorption was effective in reducing his inhibitor level, allowing him to undergo immune tolerance therapy. Costs of treatment before immunoadsorption were markedly higher than those associated with the immunoadsorption procedures (i.e., >Can 350,000 dollars and >Can 1,000,000 dollars vs. < 20,000 dollars). CONCLUSION: Immunoadsorption appears to be an effective and cost-effective alternative in the management of patients with inhibitors to FVIII.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

5/15. Resection and reconstruction of a massive femoral hemophilic pseudotumor.

    Hemophilic pseudotumor is a rare, but well-known, complication of hemophilia. We describe a 50-year-old man with mild hemophilia a, but with no previous need for factor viii supplementation, who presented with a pathologic fracture of the right femoral neck and shaft caused by a large hemophilic pseudotumor. Initial nonoperative therapy with factor replacement and skeletal traction resulted in radiographic evidence of fracture healing, but the patient's pain persisted. Therefore, he had a radical resection of his hemophilic pseudotumor (soft tissue component and entire femur), and reconstruction with a custom total femoral replacement. Six months after resection, the patient returned to full-time employment. Although pseudotumor formation is a well-recognized complication of hemophilia, the pseudotumor in our study is one of the largest yet described. More importantly, to our knowledge this is the first report of a pseudotumor treated by radical resection and reconstruction with a custom femoral prosthesis. We think that radical resection and reconstruction with a custom total femoral prosthesis is a valuable alternative to amputation in massive pseudotumors of the femur and soft tissues of the thigh.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

6/15. Use of recombinant factor viia in the management of severe bleeding episodes in patients with bernard-soulier syndrome.

    bernard-soulier syndrome (BSS) is a rare congenital platelet disorder characterized by defective platelet adhesion and manifested by spontaneous and often profuse bleeding. Recombinant factor viia (rFVIIa) is a haemostatic agent licensed for the treatment of bleeding episodes in patients with haemophilia and inhibitors, which may represent a low-risk alternative to existing therapies in the management of patients with BSS. Here, we describe the use of rFVIIa for the treatment of three severe bleeding episodes in two patients with BSS. Data were extracted by automated searching of the international, internet-based registry http://www.haemostasis.com . Patient 1, a 24-year-old woman, was admitted with severe epistaxis and hypotension. The diagnosis of BSS was confirmed by macrothrombocytopenia, absence of ristocetin-induced platelet agglutination (RIPA) and absence of glycoprotein (GP) Ibalpha and IX on the platelet surface. Epsilon aminocaproic acid (EACA; two 50-mg/kg doses), packed red blood cells (PRBCs, 2 U) and platelets (30 U) failed to control the bleeding and, after 13 h, three bolus doses of rFVIIa (90 microg/kg body weight) and a third dose of EACA were administered; bleeding stopped after the third dose of rFVIIa. Patient 2, a 15-year-old girl, initially presented with severe menorrhagia. A lack of RIPA and severe deficiency of GPIbalpha on the platelet surface confirmed the diagnosis of BSS. EACA and fresh-frozen plasma did not control the haemorrhage, but two bolus doses of rFVIIa (98 microg/kg body weight) resulted in a marked decrease in bleeding. On second admission, patient 2 had severe epistaxis and mild menorrhagia. Two rFVIIa doses (98 and 122.5 microg/kg body weight) were given, and the bleeding stopped. No adverse events were reported in these cases. These three admissions highlight the potential of rFVIIa for the treatment of severe bleeds in patients with BSS.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

7/15. Rituximab in the treatment of high responding inhibitors in severe haemophilia A.

    The development of antibodies to factor viii (FVIII) in severely affected haemophilia A patients is a serious complication associated with increased morbidity and mortality. Bypassing agents are used to treat acute bleeding episodes; however, elimination of the inhibitors can only be achieved with immune tolerance therapy (ITT) in 60-80% of cases. High responding (HR) inhibitors are more likely to respond to ITT if the titre is decreased to <5 BU over time or in selected cases after the administration of immunosuppressive drugs, plasmapheresis or immunoabsorption, techniques difficult to apply in children. Anti-CD20 (rituximab), a monoclonal antibody, was given as an alternative treatment in two haemophilic children with HR inhibitors and impaired quality of life, due to recurrent haemarthrosis. Rituximab was given at the dose of 375 mg m(-2), once weekly for four consecutive weeks. Both patients showed a partial response to rituximab reducing the inhibitor titre to <5 BU, thus facilitating ITT initiation; however, only the older patient eradicated the inhibitor within 21 days after application of ITT. The second patient, despite depletion of B cells, did not respond to ITT. No long-term side effects have been observed in both patients for a follow-up period of 20 and 18 months respectively. In conclusion, rituximab appears to be an alternative effective therapy to rapidly reduce or eliminate the inhibitor in selected cases of severely affected haemophiliacs before further proceeding to ITT. However, the dose and appropriate schedule, as well as long-term side effects need further investigation.
- - - - - - - - - -
ranking = 2
keywords = alternative
(Clic here for more details about this article)

8/15. live birth following the first mutation specific pre-implantation genetic diagnosis for haemophilia A.

    Haemophilia A is an X-linked, recessive, inherited bleeding disorder which affects 1 in 5000 males born worldwide. It is caused by mutations in the FactorVIII (F8) gene on chromosome Xq28. We describe for the first time two mutation specific, single cell protocols for pre-implantation genetic diagnosis (PGD) of haemophilia. A that enable the selection of both male and female unaffected embryos. This approach offers an alternative to sexing, frequently used for X-linked disorders, that results in the discarding of all male embryos including the 50% that would have been normal. Two families with a history of severe haemophilia. A requested carrier diagnosis and subsequently proceeded to PGD. The mutation in family 1 is a single nucleotide substitution c.5953C > T, R1966X in exon 18 and in family 2, c.5122C > T, R1689C in exon 14 of the F8 gene. Amplification efficiency was compared between distilled water and SDS/proteinase K cell lysis (98.0%, 96/98 and 80%, 112/140 respectively) using 238 single lymphocytes. blastomeres from spare IVF cleavage-stage embryos donated for research showed amplification efficiencies of 83.3% (45/54) for the R1966X and 92.9% (13/14) for the R1689C mutations. The rate of allele dropout (ADO) on heterozygous lymphocytes was 1.1% (1/93) for R1966X and 5.94% (6/101) for R1689C mutations. A single PGD treatment cycle for family 1 resulted in two embryos for transfer but these failed to implant. However, with family 2, two embryos were transferred to the uterus on day 4 resulting in a successful singleton pregnancy and subsequent live birth of a normal non-carrier female.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

9/15. Exchange transfusion and pulse steroid therapy in a hemophiliac with inhibitor.

    In thailand, the management of bleeding in hemophiliacs with inhibitor is still a challenging aspect. The source of factor viii is locally prepared fresh frozen plasma and cryoprecipitate. The factor viii concentrate and other commercial blood products are not available because of the remarkably high price. Successful management of bleeding in a 3-year-old hemophiliac boy with moderate inhibitor level (11.82 BU) was reported. He had active bleeding from a 1 cm size cut wound at upper gingivo-buccal fold and did not respond to local measures and supportive treatment. He received exchange transfusion, intravenous cyclophosphamide and high dosage of cryoprecipitate. The bleeding stopped and the inhibitor declined to 2.6 BU. A week later, the bleeding recurred with an anamnestic response of inhibitor to 5 BU. The second exchange transfusion and methylprednisolone as pulse steroid therapy were given daily for 3 days. No cryoprecipitate was infused. The bleeding gradually stopped within 48 hours and the inhibitor level declined to 3.2 BU which was still at low level for at least 2 months. Exchange transfusion and methylprednisolone as pulse steroid therapy may be an alternative treatment for controlling bleeding in a hemophiliac with moderate inhibitor level in countries where a high concentrate of factor viii or other blood products are not available. It is practical, simple, effective and of low cost.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

10/15. Extracorporeal shock-wave lithotripsy in hemophiliac patient.

    We report on a patient with hemophilia a who underwent successful extracorporeal shock-wave lithotripsy without incident. We are aware of two other reported cases in the literature. The relative risks of alternative methods of stone management and the indications and risks of blood product therapy in this condition are reviewed.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)
| Next ->


Leave a message about 'Hemophilia A'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.