Abstract:Summary. Coagulation inhibitors may occur as alloantibodies in patients with congenital factor deficiencies or as autoantibodies in patients with a previously normal coagulation. We treated 10 patients with factor VIII inhibitors (three haemophiliacs and seven patients with acquired factor VIII inhibitors) and one patient with a factor V inhibitor using extracorporeal immunoadsorption to immobilized antibodies against human immunoglobulins (Ig-Therasorb). The initial inhibitor titre was between 18 BU/ml and 54… Show more
“…Treatment with B-cell-directed antibodies such as rituximab may be an approach, but only one report has been published so far. 11 Our attempt to remove isohemagglutinins with Ig-Therasorb s , a device that has been successfully used for the removal of circulating antibodies in a variety of hematologic and immunologic diseases, 12,13 was able to remove the antibodies effectively, to restore erythropoiesis and to render the patients transfusion independent. However, our observations suggest that a complete removal of the antibodies can only be achieved by treatment of large plasma volumes at daily intervals.…”
Section: Discussionmentioning
confidence: 99%
“…11 Removal of immunoglobulins with Ig-Therasorb s immunoadsorption is a novel method for the treatment of various hematologic and immunologic diseases, such as acquired coagulation factor inhibitors, dilated cardiomyopathy and the removal of anti-HLA antibodies prior to renal transplantation. 12,13 We report our experience with Ig-Therasorb s immunoadsorption for the treatment of five patients with PRCA due to persisting isohemagglutinins after major ABO-incompatible HSCT.…”
Summary:Delayed donor red cell engraftment and prolonged red cell aplasia (PRCA) are well-recognized complications of major ABO-incompatible myeloablative and nonmyeloablative hematopoietic stem cell transplantation (HSCT). There is an intense debate about the impact on outcome, severity of hemolysis, association with graft-versus-host disease and survival after blood group-incompatible stem cell transplantation. Therefore, therapeutic strategies should be considered to avoid these possible complications. We present five patients, who received allogeneic HSCT from human leukocyte antigen-identical donors for hematological malignancies, which were treated with IgTherasorb s immunoadsorption (five treatments/week) to remove persisting incompatible isohemagglutinins. After a median of 17 treatments (range 9-25), all the patients became transfusion independent with the presentation of donor's blood group. No side effects occurred during treatment. Ig-Therasorb s immunoadsorption seems to be a promising therapeutic method for rapid, efficient and safe elimination for persisting isohemagglutinins for patients with PRCA after allogeneic hematological stem cell transplantation.
“…Treatment with B-cell-directed antibodies such as rituximab may be an approach, but only one report has been published so far. 11 Our attempt to remove isohemagglutinins with Ig-Therasorb s , a device that has been successfully used for the removal of circulating antibodies in a variety of hematologic and immunologic diseases, 12,13 was able to remove the antibodies effectively, to restore erythropoiesis and to render the patients transfusion independent. However, our observations suggest that a complete removal of the antibodies can only be achieved by treatment of large plasma volumes at daily intervals.…”
Section: Discussionmentioning
confidence: 99%
“…11 Removal of immunoglobulins with Ig-Therasorb s immunoadsorption is a novel method for the treatment of various hematologic and immunologic diseases, such as acquired coagulation factor inhibitors, dilated cardiomyopathy and the removal of anti-HLA antibodies prior to renal transplantation. 12,13 We report our experience with Ig-Therasorb s immunoadsorption for the treatment of five patients with PRCA due to persisting isohemagglutinins after major ABO-incompatible HSCT.…”
Summary:Delayed donor red cell engraftment and prolonged red cell aplasia (PRCA) are well-recognized complications of major ABO-incompatible myeloablative and nonmyeloablative hematopoietic stem cell transplantation (HSCT). There is an intense debate about the impact on outcome, severity of hemolysis, association with graft-versus-host disease and survival after blood group-incompatible stem cell transplantation. Therefore, therapeutic strategies should be considered to avoid these possible complications. We present five patients, who received allogeneic HSCT from human leukocyte antigen-identical donors for hematological malignancies, which were treated with IgTherasorb s immunoadsorption (five treatments/week) to remove persisting incompatible isohemagglutinins. After a median of 17 treatments (range 9-25), all the patients became transfusion independent with the presentation of donor's blood group. No side effects occurred during treatment. Ig-Therasorb s immunoadsorption seems to be a promising therapeutic method for rapid, efficient and safe elimination for persisting isohemagglutinins for patients with PRCA after allogeneic hematological stem cell transplantation.
“…65 The introduction of immunoadsorption techniques, including sepharose-bound staphylococcal protein A and sepharosebound polyclonal sheep antihuman antibodies, has increased the volume of plasma processed and the efficacy of the procedure. 66,67 The transitory drop of the inhibitor titer permits replacement therapy with human FVIII concentrates, which must then be administered immediately after the treatment cycle to achieve hemostasis. 68 Immunoadsorption has also been used in the setting of immune tolerance protocols (see "Immune tolerance").…”
Acquired hemophilia A is a rare bleeding diathesis caused by autoantibodies directed against clotting factor VIII and associated with an increased morbidity and mortality. This autoimmune disorder most commonly occurs in the elderly. Although it may be associated with several underlying pathologies, up to 50% of reported cases remain idiopathic. In contrast with congenital hemophilia, which is commonly characterized by hemarthroses, hemorrhages in patients with acquired hemophilia involve most frequently soft tissues. The 2 treatment priorities are to arrest the acute bleeding and to eradicate the factor VIII autoantibody. Acute bleeding episodes in patients with low-titer inhibitors can be treated using human factor VIII concentrates, whereas factor VIII bypassing agents, such as activated prothrombin complex concentrates or recombinant activated factor VII, are effective for the treatment of those with hightiter inhibitors. An analysis of the literature shows that the most effective first-line treatment for the eradication of factor VIII autoantibodies is the combination of steroids and cyclophosphamide. However, there is increasing evidence on the effectiveness of other treatment approaches, such as immune tolerance regimens and rituximab. If confirmed by large controlled studies, these innovative therapies might become a valid option for long-term eradication of factor VIII inhibitors. (Blood. 2008;112:250-255)
“…Alguns grupos têm relatado o tratamento de pacientes com altos títulos de inibidor (anti-fator V e anti-FVIII) utilizando métodos extracorpóreos de retirada de anticorpos. [52][53][54][55] Entretanto, as metodologias de retirada extracorpórea dos inibidores do FVIII não são específicas para estas imunoglobulinas e promovem a retirada de moléculas de anticorpo da circulação importantes para outras respostas imunes. Adicionalmente, em alguns casos, o indivíduo pode perder proteínas plasmáticas essenciais, como a albumina.…”
Section: Tratamento Das Crises Hemorrágicas Na Presença Do Inibidorunclassified
Dentre os distúrbios de coagulação hereditários nas populações humanas destaca-se, por sua gravidade, a hemofilia A (HA), uma coagulopatia recessiva ligada ao cromossomo X causada pela deficiência ou disfunção da glicoproteína plasmática denominada Fator VIII (FVIII). A incidência da HA pode chegar a 1:5.000 meninos nascidos vivos. Nos indivíduos com hemofilia A grave (aproximadamente 50% dos casos; atividade do FVIII<0,01 U/mL), a hemorragia pode ocorrer espontaneamente, enquanto Dias, pacientes com hemofilia moderada (cerca de 10% dos indiví-duos; atividade do FVIII 0.01-0.05 U/mL) apresentam um fenótipo intermediário. Pacientes portadores da forma leve da doença, observada em 30%-40% dos casos (atividade do FVIII 0,05-0,4 U/mL), sofrem de hemorragias pós-trauma ou pós-cirúrgicas.
REVISTA BRASILEIRA DE HEMATOLOGIA E H E M O T E R A P I A
1As anormalidades genéticas responsáveis pela hemofilia congênita incluem deleções, inversões (mais frequentemente a inversão do intron 22), mutações sem sentido, mutações de sentido trocado e pequenas deleções no
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.