Successful Treatment of Patients with Rheumatic Disorders and Acquired Factor VIII Inhibitors with Cyclophosphamide and Prednisolone Combination Therapy: Two Case Reports

Abstract

Acquired haemophilia associated with autoimmune disorders can be fatal and has been reported to be refractory to steroid therapy alone. We report two cases of female patients, aged 24 years and 54 years, with acquired haemophilia caused by factor VIII inhibitors. Underlying diseases were systemic lupus erythematosus in the 24-year-old patient and rheumatoid arthritis in the 54-year-old patient. Both conditions were nearly quiescent when the patients manifested haemorrhagic diathesis. In response to combination therapy with prednisolone and cyclophosphamide, coagulation abnormalities were resolved together with complete elimination of factor VIII inhibitors in both patients. Thus, combination therapy with alkylating agents may be recommended as initial therapy for the management of autoimmune patients with factor VIII inhibitors.

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

“…The APTT was prolonged but other laboratory screenings for hemostasis such as the platelet count and PT were normal. Similar to cases 6 and 8, the inhibiting autoantibodies could even appear in patients with quiescent SLE [9,10,12], which suggests that the appearance of the FVIII inhibitor and its level does not always correlate with underlying disease activity. We could not find any other possible inductive factor(s) for our SLE patient to develop acquired hemophilia, such as other disease conditions (including skin and respiratory diseases), infections, and use of certain drugs (including penicillin and its derivatives).…”

“…Among previous investigations of acquired hemophilia along with SLE, nine cases (including our case) are available, and the features of these reported cases are summarized in Table 1 [4][5][6][7][8][9][10][11][12]. All cases were female patients of child-bearing age with a median age of 33.9 years (range, 19-54 years).…”

Rupture of the Gallbladder in a Patient with Acquired Factor VIII Inhibitors and Systemic Lupus Erythematosus

“…Among the previous investigations of acquired factor VIII inhibitors for patients with SLE, five case reports are available (7)(8)(9)(10)(11)(12), including the present case. These patients' histories are listed in Table 2.…”

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.…”