Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review.
Hemophagocytic syndrome (HPS) is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe two cases of untreated systemic lupus erythematosus (SLE) with HPS that presented as one of the manifestations of SLE. The onset of HPS was after parturition for one patient, and after abortion for the other. Bone marrow examination revealed severe hemophagocytosis in both patients. One patient responded to pulsed methylprednisone alone, and the other responded to pulsed methylprednisone plus intravenous immunoglobulin (IVIG). We believe accurate diagnosis, intensive therapy, and sufficient supportive cares are essential in improving patients' prognosis.
Qian
Jie
J
Cheng-De Yang, Department of Rheumatology, Renji Hospital, Medical School of Shanghai Jiao Tong University, #145 Shan Dong Zhong Road, Shanghai 200001, People's Republic of China.
Yang
Cheng-De
CD
D000328:Adult; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*