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

Abstract

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.

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature

“…Antibodies are often against FVIII and are associated with a high rate of morbidity and mortality because severe bleeds occur in up to 90% of affected patients [6]. The estimated mortality rate is 7.9-22%.…”

“…Haemorrhagic events based on the production of antibodies directed against the coagulation factors are rarely observed in SLE [6]. We describe here a rare case of autoimmune hepatitis and SLE who developed acquired haemophilia A due to the presence of a high FVIII inhibitor level.…”

“…But there were isolated prolongation of the activated partial thromboplastin time (APTT), uncorrected APTT in mixing test, reduced FVIII activity (3.9%), and a high titer of factor VIII inhibitors more than 200 Bethesda Unit. Laboratory findings are listed in (Table 1) [6][7][8][9][10][11][12][13][14][15]. In physical examination, significant subcutaneous haemorrhages were found.…”

Acquired Inhibitors to Coagulation Factors in a Male Patient with Systemic Lupus Erythematosus: A Case Report and Review of the Literature

“…15,18,22 Among previous investigations of acquired hemophilia along with SLE, ten cases (including our case) are available, and the features of these reported cases are summarized in Table 3. [19][20][21][22][23][24][25][26][27][28] As reported in other similar cases, a careful follow-up of the clotting tests is important to evaluate the response to therapy and the risk of new hemorrhagic manifestations. In our case normalization of the clotting tests were achieved after 6 month since the start of treatment which was enough monotheraphy with prednisolone, although other nine similar cases were needed combination therapy for controlling the disease.…”

The treatment of hemophilia A: from protein replacement to AAV-mediated gene therapy

“…Generally, because of its X-linked and hereditary, men are affected and women are carriers. However, there are few reports about acquired hemophilia A, which is caused by suddenly appearing autoantibodies that interfere with coagulation FVIII activity (Flisiński et al 2008;Collins et al 2008;Akahoshi et al 2008;Alvarado et al 2007;Mahipal and Bilgrami 2007).…”