Abstract

A 74-years-old male who was a smoker and received treatment for hypertension, dyslipidemia, peripheral arterial disease and idiopathic interstitial pneumonia complained of subcutaneous hemorrhage of the right lower thigh. Marked anemia (hemoglobin 5.5 g/dL) and prolonged activated partial thromboplastin time (≥130 seconds) were noted. The factor VIII activity level was reduced to 1.2%, and the factor VIII inhibitor titer was 285.3 BU/mL, a diagnosis of acquired hemophilia A (AHA) was made. Then, hematomas of 5 intra-muscles were recurred. Hemostasis became difficult despite frequent and high-dose administration of recombinant human coagulation factor VIIa (total: 18 days, 305 mg). Hemostasis was achieved by switching to activated prothrombin complex concentrate (for 3 days, 18,000 units), however, cerebral infarction occurred after 36 days. After the frequent administration of bypass hemostatic agents on elderly AHA patients with several risk factors for ischemic stroke, the risk of subsequent thrombotic events may persist for 1 month.

Highlights

  • A 74-years-old Japanese male consulted a family doctor and hospitalized with subcutaneous hemorrhage with severe pain in the right lower thigh persisting for 2 days

  • Acquired hemophilia A (AHA) is an autoimmune hemorrhagic disease, whose pathogenesis involves a reduction in coagulation factor VIII (FVIII) activity related with the appearance of autoantibodies hours

  • The FVIII activity level was reduced to 1.2 % and the FVIII inhibitor titer was 285.3 Bethesda units (BU)/mL

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Summary

Introduction

A 74-years-old Japanese male consulted a family doctor and hospitalized with subcutaneous hemorrhage with severe pain in the right lower thigh persisting for 2 days. For repeated intramuscular hematomas, but cerebral infarction occurred after 36 days.

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