Acquired Hemophilia a as the Initial Hematologic Manifestation of Systemic Lupus Erythematosus in an Elderly Patient: a Rare Case Report
Year: 2026
Type: article
Study design: Case Report
Abstract: Introduction Acquired hemophilia A (AHA) is a rare coagulopathy mediated by autoantibodies against factor VIII, associated with malignancy, pregnancy, autoimmune disease, or drugs in up to half of cases. Its incidence increases with age, and its presentation with spontaneous soft-tissue bleeding, without hemarthrosis, distinguishes it from congenital hemophilia. Systemic lupus erythematosus (SLE) is a recognized autoimmune etiology, though rarely described as an initial manifestation in elderly patients. Case report A 74-year-old woman with coronary artery disease (3 prior myocardial infarctions with stenting) and diabetes developed an extensive spontaneous hematoma of the right upper limb after a routine blood draw, with edema, pain, warmth, hyporexia, and chills, without fever or trauma. On admission, prolonged aPTT (70s) with normal INR and hemoglobin of 8.9 g/dL; vascular surgery ruled out compartment syndrome. Factor VIII assay <0.4% and a positive inhibitor screen (titer 83.2 BU/mL, Bethesda method) confirmed AHA. Combined immunosuppression was started (cyclophosphamide 1000 mg IV plus corticosteroids) along with hemostatic support using activated prothrombin complex concentrate (aPCC) 4000 IU every 12 hours for 4 days, plus transfusion support. Etiologic investigation revealed antinuclear antibodies at 1:80, positive anti-double-stranded DNA antibodies, and lymphopenia. Rheumatologic evaluation supported an underlying systemic autoimmune disorder consistent with SLE (with secondary hematologic involvement), and hydroxychloroquine 400 mg/day was added. After 23 days of treatment, the inhibitor titer decreased (60.8 BU/mL) and the hematomas progressively resolved, with no new bleeding; the patient was discharged with multidisciplinary follow-up. Discussion AHA should be suspected in patients with new-onset spontaneous or disproportionate bleeding, isolated prolonged activated partial thromboplastin time with normal prothrombin time, and no previous bleeding history. Diagnostic evaluation includes a non-correcting mixing study, markedly reduced factor VIII activity, and detection and quantification of a factor VIII inhibitor by the Bethesda assay. Management requires two simultaneous approaches: rapid control of acute bleeding, usually with bypassing agents, particularly in patients with high-titer inhibitors, and eradication of the inhibitor through immunosuppressive therapy. In elderly patients, especially those with significant cardiovascular comorbidities, prohemostatic therapy requires careful assessment of thrombotic risk. In addition, the diagnosis of AHA should prompt an active investigation for associated conditions, including solid and hematologic malignancies, autoimmune diseases, pregnancy-related disorders, and potentially implicated drugs. Nevertheless, despite appropriate etiologic investigation, no underlying cause is identified in a substantial proportion of cases, particularly among older patients, and these cases are classified as idiopathic AHA. Conclusion This case underscores the importance of including acquired hemophilia in the differential diagnosis of spontaneous bleeding in elderly patients without a prior bleeding history, highlighting SLE as an etiology to investigate even in age groups atypical for its initial diagnosis. It illustrates successful management through combined hemostatic and immunosuppressive therapy.
Institutions: Universidade de São Paulo, Hospital Felício Rocho
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W7223155685