Woo P, Pereira R S, Lever A M
J Rheumatol. 1984 Dec;11(6):828-31.
A homozygous C2 deficient patient with a lupus-like syndrome developed hypogammaglobulinemia soon after treatment with prednisolone together with phenytoin, replaced subsequently by carbamazepine. She suffered from recurrent chest infections and her lupus symptoms continued unabated. In vitro tests of immunoglobulin production by her Epstein Barr virus transformed B cells showed typical patterns of reduced IgA and IgG production seen in common variable hypogammaglobulinemia. An opsonisation defect to Hemophilus influenzae was also demonstrated which could be reversed by the addition of pooled human gammaglobulin. Serum IgM and IgG levels returned to normal 2 years after withdrawal of phenytoin and prednisolone, but 3 years later, she remained IgA deficient and the in vitro abnormalities persisted.
一名患有狼疮样综合征的纯合子C2缺陷患者在接受泼尼松龙和苯妥英钠治疗后不久出现低丙种球蛋白血症,随后苯妥英钠被卡马西平取代。她反复出现胸部感染,狼疮症状持续未减。对其爱泼斯坦-巴尔病毒转化的B细胞进行免疫球蛋白产生的体外试验显示,出现了常见可变型低丙种球蛋白血症中典型的IgA和IgG产生减少模式。还证实了对流感嗜血杆菌的调理缺陷,添加混合人丙种球蛋白后该缺陷可得到逆转。停用苯妥英钠和泼尼松龙2年后,血清IgM和IgG水平恢复正常,但3年后,她仍存在IgA缺陷,体外异常情况持续存在。