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Classic Polyarteritis Nodosa Presenting Rare Clinical Manifestations in a Patient with Hemophilia A

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Abstract

A 35-year-old patient with hemophilia A presented with rapidly progressive polyarteritis nodosa (PAN). He had been infected with hepatitis B virus (HBV) by repeated transfusion and was positive for hepatitis B surface antigen but negative for hepatitis B surface antibody.The patient presented symptoms of acute epididymitis followed by emergency admission because of acute appendicitis. On day 7 of admission, he complained of severe back pain, and computerized tomography (CT) showed massive perirenal hematoma. On day 49, mild monoplegia in the left arm suddenly developed, and CT and magnetic resonance imaging revealed multiple cerebral infarctions. Factor VIII replacement therapy was attenuated; however, cerebral infarction was progressive and extended throughout the cerebral hemispheres. He was diagnosed with classic polyarteritis nodosa (cPAN), and pulse methylprednisolone was continued.The patient died of supratentorial herniation, and autopsy revealed that vasculitis associated with intimal thickening was present in the liver, pancreas, intestine, kidneys, and larger-sized cerebral arteries. The development of cPAN appeared to have originated from chronic HBV infection, and this is the first report of cPAN in hemophilia patients. Concomitant hemorrhagic and thrombotic manifestations of cPAN are hardly treatable in patients with coagulation disorders, and the current case may represent a rare transfusion-related complication in hemophilia patients.

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Authors and Affiliations

  1. Department of Hematology, Nagoya University School of Medicine, 65 Tsurumai-cho, Showa-ku, 466-8560, Nagoya, Japan

    Tadashi Matsushita, Tatsuya Adachi & Tomoki Naoe

  2. Division of Neurology, Department of Clinical Neurosciences, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Hiroaki Adachi, Hidetaka Watanabe & Gen Sobue

  3. Department of Pathology, Nagoya University Hospital, Nagoya, Japan

    Yoshie Shimoyama

  4. Department of Pathology, Japanese Red Cross Nagoya First Hospital, Nagoya, Japan

    Masafumi Ito

  5. Department of Medical Technology, Nagoya University School of Health Sciences, Nagoya, Japan

    Tetsuhito Kojima

  6. Nagoya Medical Center, Nagoya, Japan

    Hidehiko Saito

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  1. Tadashi Matsushita
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Correspondence to Tadashi Matsushita.

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Matsushita, T., Adachi, H., Watanabe, H. et al. Classic Polyarteritis Nodosa Presenting Rare Clinical Manifestations in a Patient with Hemophilia A. Int J Hematol 83, 420–425 (2006). https://doi.org/10.1532/IJH97.05185

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  • DOI: https://doi.org/10.1532/IJH97.05185

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