Effective and safe use of recombinant factor VIIa (NovoSeven<sup>®</sup>) in elderly mild haemophilia A patients with high‐titre antibodies against factor VIII

Leebeek, Frank W.G.; Kappers‐Klunne, M. C.; Jie, K. S. G.

doi:10.1111/j.1365-2516.2004.00882.x

Cited by 17 publications

(13 citation statements)

References 13 publications

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“…Bleeding episodes in patients with mild/moderate hemophilia and inhibitors are usually treated with bypassing agents, such as APCC or rFVIIa [152,153]. Some patients with inhibitors, especially those with basal measurable FVIII levels, can be treated successfully with DDAVP as the antibody may or may not inhibit endogenous FVIII to the same extent as exogenous FVIII [138].…”

Section: Treatmentmentioning

confidence: 99%

Mild hemophilia A

Franchini

Favaloro

Lippi

2010

Journal of Thrombosis and Haemostasis

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Summary. Mild hemophilia A (HA), defined by clinical features and factor VIII coagulant activity (FVIII:C) between 0.05 and 0.40 IU mL−1, is characteristically distinct from severe HA. Indeed, although the molecular characterization of mild HA has permitted the identification of specific underlying mutations, its clinical phenotype is strikingly different from that of patients with a severe FVIII defect, where spontaneous hemorrhages or recurrent joint bleeding are usual manifestations. With aging, mild HA patients may develop complications (i.e. cancers and cardiovascular disorders), the management of which may prove challenging due to the concomitant bleeding tendency. Furthermore, the development of inhibitors provides an additional major complication in these patients, because it increases the severity of the bleeding phenotype and complicates their management. Standard management of mild HA includes the use of desmopressin and antifibrinolytic agents for minor bleeding episodes or surgical procedures, whilst major bleeding or surgery requires replacement therapy with FVIII concentrates. As regards treatment of patients with inhibitors, bypassing agents (i.e. activated prothrombin complex concentrates and recombinant activated FVII) have proven effective in the treatment of bleeding episodes, but as there are insufficient data to determine the optimal approach to immune tolerance induction in this group of patients, their optimal management remains controversial. Rituximab is a newer, promising therapeutic option for inhibitor eradication in such patients. Many aspects concerning mild HA remain to be clarified, including the molecular basis, the natural history and the optimal diagnostic and therapeutic strategies. Only large prospective studies will shed light on this condition.

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Section: Treatmentmentioning

confidence: 99%

Mild hemophilia A

Franchini

Favaloro

Lippi

2010

Journal of Thrombosis and Haemostasis

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“…There are concerns regarding the use of bypassing agents in these elderly patients considered at risk of thrombotic complications. Leebeek et al (2004) recently documented the safe and effective use of rFVIIa in three elderly patients with mild haemophilia A and high‐titre inhibitors. An additional case was recently described by Rivolta et al (2009a,b), who successfully used rFVIIa in a 70‐year‐old haemophilia A patient who had both a high titre inhibitor, causing anaphylactoid reactions and coronary artery disease.…”

Section: Clinical Management Of Co‐morbidities In Elderly Haemophiliamentioning

confidence: 99%

Co‐morbidities and quality of life in elderly persons with haemophilia

Franchini¹,

Mannucci

2010

Br J Haematol

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SummaryThe life expectancy and quality of life of persons with haemophilia has dramatically increased since the 1970s, with the exception of the increased rate of deaths observed during the 1980s and the 1990s due to blood-borne viral infections. Improvements of factor replacement therapy, treatment of infectious diseases and comprehensive health care provided by specialised haemophilia centres are the main determinants of the increasing age of the haemophilia population. As a consequence, a growing number of these patients develop age-related co-morbidities, such as cardiovascular disease and cancer. The care of these previously rare conditions is a new challenge for caregivers in haemophilia centres. This review focuses on co-morbidities in the ageing haemophilia patients, their impact on quality of life and their complex management.

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“…Treatment modalities for AH include activated prothrombin complex concentrate and activated recombinant factor VII. 25,49 In addition, immunosuppressive therapy is also useful in reducing the production of factor VIII inhibitors such as prednisolone, cyclophosphamide and cyclosporine. 21,26,50,51 Rituximab (anti-CD20 monoclonal antibody) has also been tried and reported effective.…”

Section: Discussionmentioning

confidence: 99%

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

Arakaki

Yamamoto

Awazawa

et al. 2008

The Journal of Dermatology

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Linear immunoglobulin (Ig)A bullous dermatosis is a rare autoimmune subepidermal bullous dermatosis caused by circulating IgA autoantibodies directed against the antigens at the basement membrane zone. Most linear IgA bullous dermatosis cases are idiopathic, but some are associated with the use of certain drugs, infections, lymphoproliferative disorders, internal malignancies, autoimmune disorders, collagen diseases or, very rarely, other skin diseases, including autoimmune bullous diseases. Acquired hemophilia is also rare; it is a coagulation disease caused by anti-factor VIII IgG antibodies. Acquired hemophilia has been reported to be associated with malignant tumors, pregnancy or postpartum, drug reactions, collagen diseases such as rheumatoid arthritis, autoimmune disorders, and skin diseases such as psoriasis and pemphigus. We report a case of hemophilia acquired during the course of linear IgA bullous dermatosis and review reported cases of autoimmune bullous dermatoses associated with acquired hemophilia.

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Effective and safe use of recombinant factor VIIa (NovoSeven^®) in elderly mild haemophilia A patients with high‐titre antibodies against factor VIII

Cited by 17 publications

References 13 publications

Mild hemophilia A

Mild hemophilia A

Co‐morbidities and quality of life in elderly persons with haemophilia

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

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Effective and safe use of recombinant factor VIIa (NovoSeven®) in elderly mild haemophilia A patients with high‐titre antibodies against factor VIII

Cited by 17 publications

References 13 publications

Mild hemophilia A

Mild hemophilia A

Co‐morbidities and quality of life in elderly persons with haemophilia

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

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Product

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Effective and safe use of recombinant factor VIIa (NovoSeven^®) in elderly mild haemophilia A patients with high‐titre antibodies against factor VIII