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Mycobacterium avium complex-associated hemophagocytic syndrome in systemic lupus erythematosus patient: report of one case

Department of Pediatrics, Veterans General Hospital-Taichung, Taiwan, Republic of China

L-S Fu

Department of Pediatrics, Veterans General Hospital-Taichung, Taiwan, Republic of China, lsfu{at}vghtc.vghtc.gov.tw

J-L Lan

Department of Allergy-Immunology-Rheumatology, Veterans General Hospital-Taichung, Taiwan, Republic of China, School of Medicine, Taipei Medical University, Taipei, Republic of China

G-H Shen

Department of Chest Medicine, Veterans General Hospital-Taichung, Taiwan, Republic of China

G Chou

Department of Pathology, Veterans General Hospital-Taichung, Taiwan, Republic of China

C-F Tseng

Department of Pediatrics, Veterans General Hospital-Taichung, Taiwan, Republic of China

C-S Chi

Department of Pediatrics, Veterans General Hospital-Taichung, Taiwan, Republic of China

Hemophagocytic syndrome (HPS) in systemic lupus erythematosus(SLE) patients has not commonly been reported. In this case study, we report the first case of Mycobacterium avium complex (MAC)-associated hemophagocytic syndrome in a patient with systemic lupus erythematosus (SLE). This SLE patient, a 15-year-old girl, had been on a high dose of prednisolone (> 0.5 mg=kg=day) for more than 3 years. She presented with a spiking fever, hepatosplenomegaly, pancytopenia, hyperferritinemia and adult respiratory distress syndrome. Bone marrow examination revealed hemophagocytosis as well as non-caseating granulomatosis. There was no indication of SLE flare-up. She responded poorly to initial treatment with methyl-prednisolone, intravenous immunoglobulin, etoposide, and drugs for Mycobacterium tuberculosis including rifampin, ethambutol, isoniazid and pyramide. However, gastric lavage culture revealed MAC. Following treatment with clarithromycin, ciprofloxacin and amikacin, her condition gradually improved and she was discharged 3 months after admission. In SLE patients with pancytopenia and hyperferritinemia, MAC-associated HPS should be considered in the differential diagnosis.

Key Words: hemophagocytic syndrome • Mycobacterium avium complex • systemic lupus erythematosus

Lupus, Vol. 12, No. 4, 312-316 (2003)
DOI: 10.1191/0961203303lu326cr


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