Cyclosporin treatment of a woman with acquired haemophilia due to factor VIII:C inhibitor

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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“…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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“…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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“…[34][35][36][37][38][39] Cyclosporine (CsA) has also been used for inhibitor suppression. [40][41][42][43] Use of CsA requires monitoring drug levels to ensure efficacy and prevent toxicity. However, its use is limited due to availability of other strategies such as cytotoxic drugs and Rituximab.…”

Section: Discussionmentioning

confidence: 99%