The Development of Acute Systemic Multiple Thrombosis after Achieving Remission during Systemic Glucocorticoid Therapy for Acquired Hemophilia A

Kazuhiro Toyama; Atsushi Yasumoto; Fumihiko Nakamura; Shunya Arai; Mineo Kurokawa

doi:10.2169/internalmedicine.0450-17

CASE REPORTS

The Development of Acute Systemic Multiple Thrombosis after Achieving Remission during Systemic Glucocorticoid Therapy for Acquired Hemophilia A

Kazuhiro Toyama, Atsushi Yasumoto, Fumihiko Nakamura, Shunya Arai, Mineo Kurokawa

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Keywords: acquired hemophilia A, thrombosis, glucocorticoid therapy, von Willebrand factor

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 15 Pages 2237-2241

DOI https://doi.org/10.2169/internalmedicine.0450-17

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Abstract

Acquired hemophilia A (AHA) is a hemorrhagic disorder. Whether or not severe thrombotic events can develop without the use of bypassing agents in AHA patients is unclear. An 80-year-old woman with AHA underwent immunosuppressive therapy with prednisolone at 1 mg/kg daily. After achieving remission, she suddenly developed multiple organ failure due to acute systemic thrombosis and died within a few hours of the diagnosis. Patients with AHA, especially those with risk factors for thrombosis, have a considerable risk of developing thrombosis during the recovery phase of factor VIII activity and should be carefully monitored by coagulation testing.

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