title: Classic polyarteritis nodosa presenting rare clinical manifestations in a patient with hemophilia A.
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.
authors.affiliation: Department of Hematology, Nagoya University Graduate School of Medicine, Nagoya, Japan. tmatsu@med.nagoya-u.ac.jp
authors.forename: Tadashi
authors.initials: T
authors.lastname: Matsushita
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Adachi
authors.forename: Hidetaka
authors.initials: H
authors.lastname: Watanabe
authors.forename: Yoshie
authors.initials: Y
authors.lastname: Shimoyama
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Adachi
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
authors.forename: Masafumi
authors.initials: M
authors.lastname: Ito
authors.forename: Tetsuhito
authors.initials: T
authors.lastname: Kojima
authors.forename: Hidehiko
authors.initials: H
authors.lastname: Saito
authors.forename: Tomoki
authors.initials: T
authors.lastname: Naoe
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D006467:Hemophilia A / Q000150:complications* / Q000473:pathology / Q000628:therapy / Q000821:virology; D019694:Hepatitis B, Chronic / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D010488:Polyarteritis Nodosa / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology / Q000821:virology; D065227:Transfusion Reaction