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

Autor: Lan Jl, Chou G, Chi Cs, Fu Ls, Yang Wk, Tseng Cf, Shen Gh
Rok vydání: 2003
Předmět:
Zdroj: Lupus. 12:312-316
ISSN: 1477-0962
0961-2033
DOI: 10.1191/0961203303lu326cr
Popis: 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.
Databáze: OpenAIRE