Arai J, Yasukawa M, Takada K, Tange Y, Saiki O, Horiuchi T, Tamai T, Fujita S
First Department of Internal Medicine, Ehime University School of Medicine, Japan.
Clin Exp Rheumatol. 1998 Jan-Feb;16(1):84-6.
A 24-year-old woman had suffered from recurrent bacterial infections and clinical manifestations of systemic lupus erythematosus (SLE). Laboratory findings disclosed an elevated level of serum IgM, markedly decreased IgG, IgA, IgD and IgE levels, and low levels of serum complement. Both the CD40 and CD40 ligands appeared to be normally expressed. Assays of in vitro immunoglobulin production by lymphocytes showed that IgM was produced normally and that IgE but not IgG or IgA production was rescued by signaling through CD40 on B cells. The proliferative response of lymphocytes to phobol ester was markedly decreased, suggesting some impairment of signal transduction in the patient's lymphocytes.
一名24岁女性反复出现细菌感染，并伴有系统性红斑狼疮（SLE）的临床表现。实验室检查发现血清IgM水平升高，IgG、IgA、IgD和IgE水平明显降低，血清补体水平也较低。CD40和CD40配体的表达似乎正常。淋巴细胞体外免疫球蛋白产生检测显示，IgM产生正常，通过B细胞上的CD40信号传导可挽救IgE而非IgG或IgA的产生。淋巴细胞对佛波酯的增殖反应明显降低，提示患者淋巴细胞的信号转导存在某种损伤。
