Inhibitors: resolving diagnostic and therapeutic dilemmas

DiMichele, Donna

doi:10.1046/j.1365-2516.2002.00626.x

Cited by 137 publications

(144 citation statements)

References 49 publications

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“…1 The development of high-titer factor VIII inhibitors (>5 Bethesda units [BU]) complicates treatment because bleeding no longer responds to standard factor VIII replacement. 2,3 Alternative forms of clottingfactor concentrates, known as bypassing agents, are used to treat bleeding in these patients.…”

Section: Discussionmentioning

confidence: 99%

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

et al. 2011

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Section: Discussionmentioning

confidence: 99%

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

et al. 2011

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“…In accord with our findings are observations in patients with hemophilia A and B in whom null alleles of factor VIII and IX, respectively, were associated with a high frequency of inhibitor development. 21,22 HLA class II subgroups were suggested to be a weak determinant for development of an inhibitor to factor VIII in hemophilia A. 23 All patients were exposed to plasma products.…”

Section: Discussionmentioning

confidence: 99%

Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency

Salomon

Zivelin²,

Livnat³

et al. 2003

Blood

120

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Factor XI deficiency, an injury-related bleeding disorder, is rare worldwide but common in Jews in whom 2 mutations, Glu117Stop (type II) and Phe283Leu (type III), prevail. Mean factor XI activities in homozygotes for Glu117Stop and for Phe283Leu are 1 and 10 U/dL, respectively. Inhibitors to factor XI in patients with severe factor XI deficiency have been reported in a small number of instances. This study was undertaken to determine the prevalence of acquired inhibitors against factor XI in patients with severe factor XI deficiency, discern whether these inhibitors are related to specific mutations, and characterize their activity. Clinical information was obtained from unrelated patients with severe factor XI deficiency, and blood was analyzed for factor XI activity, inhibitor to factor XI, and causative mutations. Immunoglobulin G purified from patients with an inhibitory activity was tested for binding to factor XI, effects on activation of factor XI by factor XIIa and thrombin, and activation of factor IX by exogenous factor XIa. Of 118 Israeli patients, 7 had an inhibitor; all belonged to a subgroup of 21 homozygotes for Glu117Stop who had a history of plasma replacement therapy. Three additional patients with inhibitors from the United Kingdom and the United States also had this genotype and were exposed to plasma. The inhibitors affected factor XI activation by thrombin or factor XIIa, and activation of factor IX by factor XIa.

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“…Em resumo, as condições preeexistentes as quais parecem influenciar o desenvolvimento de anticorpos incluem: o tipo e a gravidade da hemofilia, a etnia do paciente, o genótipo da hemofilia e o imunofenótipo. 15 A predisposição genética para o desenvolvimento de inibidores anti-FVIII é demonstrada por estudos familiares. Nos regis-tros da MIBS (Malmö International Brother Study) foram encontrados mais membros de uma mesma família com inibidor do que poderia ser explicado por uma chance casual.…”

Section: Natureza Dos Anticorpos Inibidores Do Fviiiunclassified

“…Especificamente, é bastante debatido os riscos relativos do FVIII recombinante ou derivado do plasma para induzir a formação do anticorpo. 15 Recentemente, um estudo mostrou que o uso terapêutico de FVIII recombinante aumenta em cerca de 2,4 vezes o risco de desenvolver inibidores se comparado com o uso de FVIII derivado do plasma. 21 Outras situações ambientais devem modificar a predisposição genética para desenvolver o inibidor: o local de hemorragias frequentes; a coexistência de inflamação; a intensidade de reposição do fator; o estado nutricional.…”

Section: Natureza Dos Anticorpos Inibidores Do Fviiiunclassified

“…21 Outras situações ambientais devem modificar a predisposição genética para desenvolver o inibidor: o local de hemorragias frequentes; a coexistência de inflamação; a intensidade de reposição do fator; o estado nutricional. 15 Adicionalmente, o risco de desenvolver inibidores aumenta com o início precoce de terapia com FVIII.…”

Section: Natureza Dos Anticorpos Inibidores Do Fviiiunclassified

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Desenvolvimento de inibidores do fator VIII na hemofilia A

Chaves¹,

Rodrigues²

2009

Rev. Bras. Hematol. Hemoter.

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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

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Inhibitors: resolving diagnostic and therapeutic dilemmas

Cited by 137 publications

References 49 publications

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency

Desenvolvimento de inibidores do fator VIII na hemofilia A

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