Letter to the Editor
Pregnancy exacerbates complications of acquired hemophilia in a patient with systemic lupus erythematosus

Acquired hemophilia (AH) is a rare disease characterized by the production of antibodies directed against blood coagulation fVIII leading to coagulation disorders due to lowering of its activity. These are mostly the IgG class of antibodies which does not fixate the complement (IgG1 and IgG4 class) against A2 or C2 domains of fVIII which are responsible for its activity. The incidence of AH is 1.3–1.5 cases per 1 000 000 population per year and may affect both women and men [1]. Acquired hemophilia may be a primary disease or occur in the course of malignant diseases (e.g. prostate and lung cancer, lymphoproliferative and myeloproliferative syndromes), autoimmune diseases, during drug therapy (e.g. penicillin, sulfonamides, phenytoin, chloramphenicol, methyldopa, fludarabine and interferon) and pregnancy and puerperium. There were reported cases of AH in the course of systemic lupus erythematosus (SLE), primary Sjögren’s syndrome, rheumatoid arthritis, giant cell arteritis and polymyositis. In around 50% of cases the exact cause is unknown [2, 3]. Acquired hemophilia can occur at any age and is characterized by hemorrhagic diathesis caused by bleeding into soft tissues, skin and mucosa. Other symptoms include large areas of ecchymoses, hematomas under the skin, nosebleed, hemoptysis, vaginal, urinal, gastrointestinal and retroperitoneal bleeding. In contrast to congenital hemophilia, hemarthroses are rare [2]. Laboratory tests reveal prolonged activated partial thromboplastin time (APTT), which is not corrected by infusion of fresh frozen plasma (FFP) and prothrombin time (PT) within normal range. Acquired hemophilia is usually characterized by a severe course of the disease and a high mortality rate [4, 5].
A 25-year-old woman with suspected antiphospholipid syndrome (APS) in the course of SLE was admitted to the Department of Rheumatology and Internal Medicine in September 2010 to assess the disease course and treatment options. A detailed history revealed recurrent episodes of urticaria and peripheral arteritis since 2007. Since then, she has been under the dermatologist’s supervision and laboratory tests revealed antinuclear antibodies (ANA), decreased levels of both complement C3 and C4 components, normocytic anemia and leukopenia. The diagnosis of SLE was established on the basis of both clinical and laboratory findings and glucocorticoid (GC) therapy was introduced. The treatment consisted of five pulses of intravenous methylprednisolone...


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