Scandinavian Journal of Rheumatology · 2005 · 10 citations · 11 references
Life SciencesImmunologyInfectious DiseasesHematopathologyImmunopathologyMedical MicrobiologyAutoimmune DiseasesAutoimmune Hematologic DiseaseHematologic DisordersHematologic PathologyHistiocytic DisordersLiver DiseaseBehçet Disease Oral UlcerationChronic Oral UlcersDna In Situ HybridizationEnzymesGenital UlcersHemophagocytosis In Bone MarrowOral Mucosal UlcersSteroid Therapy Management
We present a case of Epstein-Barr virus (EBV)-associated haemophagocytic syndrome in a patient with Behçet's disease. A 43-year-old man, who had been receiving treatment under the diagnosis of Behçet's disease for recurrent oral ulcers, genital ulcer, ileal ulcer, and arthritis, had been admitted for fever, headache, and nausea developed 3 days ago. Laboratory data showed pancytopaenia, an increase in liver enzymes, lactate dehydrogenase (LDH) and ferritin. Haemophagocytic syndrome was diagnosed from histiocytosis and haemophagocytosis by macrophages, shown in the bone marrow aspiration and biopsy, and in situ hybridization for EBV showed a positive finding. The patient recovered rapidly after steroid therapy. This is the first report of EBV-associated haemophagocytic syndrome developed in a patient with Behçet's disease.
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Macrophage activation syndrome and rheumatic disease in childhood: a report of four new cases.
JL Stéphan, Jason Zeller, P. Hubert et al. · PubMed · 1993 · 186 citations
Life Sciences, Immunology, Pathology +11
Etanercept in the treatment of macrophage activation syndrome.
S Prahalad, Kevin E. Bove, David S. Dickens et al. · PubMed · 2001 · 142 citations