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 pr...

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Bibliographic Details
Published in:Rheumatology international 2006-04, Vol.26 (6), p.573-576
Main Authors: HAYAKAWA, Ikuko, SHIRASAKI, Fumiaki, IKEDA, Hiroko, OISHI, Naoto, HASEGAWA, Minoru, SATO, Shinichi, TAKEHARA, Kazuhiko
Format: Article
Language:English
Subjects:
Aged
Antiviral Agents - administration & dosage
Biological and medical sciences
Blood Transfusion
Bone marrow
Diseases of the osteoarticular system
Epstein-Barr Virus Infections - therapy
Etoposide - administration & dosage
Fatal Outcome
Female
Glucocorticoids - administration & dosage
Herpesvirus 4, Human - genetics
Humans
Immunoglobulins, Intravenous - administration & dosage
Immunologic Factors - administration & dosage
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - therapy
Medical research
Medical sciences
Methylprednisolone - administration & dosage
Polyarteritis Nodosa - complications
Polyarteritis Nodosa - diagnosis
Prednisolone - administration & dosage
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
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Summary: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.
ISSN:0172-8172
1437-160X
DOI:10.1007/s00296-005-0024-0