Acquired haemophilia A: Successful treatment with immunosuppression, methylprednisolone pulses and oral cyclosporin

Pardos-Gea, José; Ordi-Ros, José; Altisent, Carmen; Balada, Eva; Pérez‐López, Jorge F.; Vilardell, M

doi:10.1160/th04-12-0827

Cited by 14 publications

(16 citation statements)

References 14 publications

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“…A number of cases have been reported in which ciclopsorin A has induced CR following failed first line therapy (Hart et al , 1988; Pfliegler et al , 1989; Schulman et al , 1996; Au et al , 2004; Pardos‐Gea et al , 2006).…”

Section: Inhibitor Eradicationmentioning

confidence: 99%

“…There is no published evidence to support its use in patients with higher titre inhibitors as suggested by some authors (Aggarwal et al, 2006). Ciclosporin A A number of cases have been reported in which ciclopsorin A has induced CR following failed first line therapy (Hart et al, 1988;Pfliegler et al, 1989;Schulman et al, 1996;Au et al, 2004;Pardos-Gea et al, 2006).…”

Section: Rituximabmentioning

confidence: 99%

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Advances in the understanding of acquired haemophilia A: implications for clinical practice

2009

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SummaryAcquired haemophilia A is an auto-immune disease caused by an inhibitory antibody to factor VIII. Patients with an acquired factor VIII inhibitor are at risk of life-and limb-threatening bleeding until the inhibitor has been eradicated. Management relies on rapid and accurate diagnosis, control of bleeding episodes, investigation for a precipitating cause and eradication of the inhibitor by immunosuppression. Patients should always be managed jointly with a specialist centre even if they present without overt bleeding. Despite an extensive literature, few controlled data are available and management guidelines are predominantly based on case reports, retrospective cohorts and expert opinion. This paper reviews the current literature on incidence, pathogenesis, diagnosis, haemostatic therapy and inhibitor eradication strategies. Potential future developments are discussed.

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Section: Inhibitor Eradicationmentioning

confidence: 99%

Section: Rituximabmentioning

confidence: 99%

Advances in the understanding of acquired haemophilia A: implications for clinical practice

2009

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“…A number of cases have been reported in which cyclosporin A has induced CR after failed first‐line therapy [31,40,47,51,62].…”

Section: Inhibitor Eradicationmentioning

confidence: 99%

Treatment of acquired hemophilia A

Collins

2007

Journal of Thrombosis and Haemostasis

106

118

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Summary. Acquired hemophilia A (AH) is an autoimmune disease that leads to potentially severe bleeding. Management relies on rapid and accurate diagnosis, control of bleeding episodes and eradication of the inhibitor by immunosuppression. There is extensive literature about the disease but only few controlled data are available. This paper reviews the current literature on treatment strategies for hemostatic therapy and inhibitor eradication. Potential future developments are discussed.

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“…Previous studies in a prospective randomized trial have shown that CY is effective as a second-line therapy for many of those who are steroid-resistant [13], and that successful treatment and long-lasting remission was achieved in SLE patients with acquired FVIII inhibitors [8,11]. On the other hand, CsA, alone or with PSL as salvage therapy or as a first-line treatment, has been shown to be effective for acquired hemophilia [23][24][25][26][27][28][29]. It can be administered to patients who do not respond to standard immunosuppressive regimens and is also effective in patients with underlying SLE [3,6,12], such as in our case.…”

Section: Discussionmentioning

confidence: 99%

Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review

Akahoshi¹,

Aizawa²,

Nagano³

et al. 2008

Mod Rheumatol

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We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) who developed acquired hemophilia caused by factor VIII (FVIII) inhibitors. She manifested spontaneous bleeding symptoms such as ecchymoses and hematuria. Laboratory findings showed an isolated prolongation of the activated partial thromboplastin time, reduced FVIII activity, and a high titer of FVIII inhibitors. She was successfully treated with oral predonisolone and cyclosporine in combination with steroid and cyclophosphamide pulse therapy.

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Acquired haemophilia A: Successful treatment with immunosuppression, methylprednisolone pulses and oral cyclosporin

Cited by 14 publications

References 14 publications

Advances in the understanding of acquired haemophilia A: implications for clinical practice

Advances in the understanding of acquired haemophilia A: implications for clinical practice

Treatment of acquired hemophilia A

Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review

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