The management of acquired haemophilia

GREEN, D.

doi:10.1111/j.1365-2516.2006.01383.x

Cited by 25 publications

(19 citation statements)

References 34 publications

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“…[1][2][3]5,6,[19][20][21][22][23][24] Clinical bleeding at presentation was observed in 96.2% of patients and was severe in 69.5%. Severity of bleeding is widely variable, and it is recognized that many patients do not require hemostatic treatment.…”

Section: Discussionmentioning

confidence: 99%

Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

Baudo

Collins

Huth‐Kühne³

et al. 2012

Blood

338

457

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Section: Discussionmentioning

confidence: 99%

Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

Baudo

Collins

Huth‐Kühne³

et al. 2012

Blood

338

457

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“…The choice of the most appropriate therapeutic strategy will depend on the site and severity of the bleeding and the inhibitor titer. 37 Of note, any potential additional risk situation for bleeding, such as intramuscular injections, invasive procedures, or the use of antiplatelet agents, should also be avoided.…”

Section: Treatment Of Acute Hemorrhagesmentioning

confidence: 99%

Acquired factor VIII inhibitors

Franchini

Lippi

2008

Blood

248

321

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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)

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“…Patients with AHA are often elderly; co-morbidities and co-medications such as anti-platelet agents may also influence the clinical profile and require an individualized therapeutic approach. 5,6 There are few clinical parameters that can be used to guide patient management because the bleeding phenotype rarely correlates with laboratory assessments. 1 In contrast to congenital hemophilia, no high-level evidence exists to support treatment recommendations for patients with AHA.…”

Section: Introductionmentioning

confidence: 99%