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전신홍반루푸스에 동반된 이차성 혈구포식성 림프조직구증 3예
임은수 ( Eunsoo Lim ),김영건 ( Young Geon Kim ),최원선 ( Won Sun Choi ),정유석 ( Yu Soek Jung ),한재호 ( Jae Ho Han ),배창범 ( Chang Bum Bae ),정주양 ( Ju Yang Jung ),김현아 ( Hyoun Ah Kim ),서창희 ( Chang Hee Suh ) 대한류마티스학회 2015 대한류마티스학회지 Vol.22 No.3
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hyperferritinemia, and phagocytosis of hematopoietic cells in bone marrow, liver, or lymph nodes. HLH can occur during the course of systemic lupus erythematosus (SLE), but can also be a presenting manifestation. Because development of pancytopenia occurs in less than 10 percent of SLE cases, investigation for HLH is necessary when otherwise unexplained pancytopenia persists despite adequate treatment. We experienced three cases of secondary HLH associated with SLE. Among the three patients, two patients developed HLH during the clinical course of SLE. The other patient who presented with pancytopenia was first diagnosed with HLH, and later with SLE. In her case, HLH turned out to be a presenting manifestation of SLE. We report on three successfully treated cases, and discuss the prevalence, characteristics, treatments, and prognosis of secondary HLH associated with SLE. (J Rheum Dis 2015;22:180-185)