Reactive hemophagocytic syndrome in a patient with polyarteritis nodosa associated with Epstein-Barr virus reactivation.
A 73-year-old woman with erythematous nodules was admitted to our hospital in December 2003. She was diagnosed with polyarteritis nodosa (PAN) from skin biopsy and laboratory data. Following the treatment with oral prednisolone (40 mg/day), her condition improved. Four days after the reduction of prednisolone, she became febrile. Bone marrow aspiration revealed an increase in the number of marrow macrophages, and phagocytosis of blood cells. The Epstein-Barr virus genome was detected in her peripheral blood. A diagnosis of hemophagocytic syndrome was made. Moreover, intestinal bleeding developed and the patient was given medical treatment consisting of methylprednisolone pulse therapy, intravenous immunoglobulin, weekly intravenous VP-16, and several blood transfusions. In addition, embolization of a branch of the ileal artery was performed. Despite the above treatments, the patient died. Autopsy revealed hemophagocytosis in bone marrow and perforation of ileocecal region. This case suggests that risks for hemophagocytic syndrome in PAN patients should be recognized.
Hayakawa
Ikuko
I
Department of Dermatology, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa Ishikawa, 920-8641, Japan.
Shirasaki
Fumiaki
F
Ikeda
Hiroko
H
Oishi
Naoto
N
Hasegawa
Minoru
M
Sato
Shinichi
S
Takehara
Kazuhiko
K
D000368:Aged; D000998:Antiviral Agents / Q000008:administration & dosage; D001803:Blood Transfusion; D020031:Epstein-Barr Virus Infections / Q000628:therapy*; D005047:Etoposide / Q000008:administration & dosage*; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D004854:Herpesvirus 4, Human / Q000235:genetics*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000628:therapy*; D008775:Methylprednisolone / Q000008:administration & dosage*; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000175:diagnosis; D011239:Prednisolone / Q000008:administration & dosage
D000998:Antiviral Agents; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D005047:Etoposide; D011239:Prednisolone; D008775:Methylprednisolone