Mayumi M, Yamaoka K, Tsutsui T, Mizue H, Doi A, Matsuyama M, Ito S, Shinomiya K, Mikawa H
Eur J Pediatr. 1986 Apr;145(1-2):99-103. doi: 10.1007/BF00441866.
A 16-year-old girl with disseminated molluscum contagiosum (MC) was found to have a very low level of serum IgM, elevated levels of IgG and IgA, and a high level of IgE. She had normal numbers of peripheral blood IgM+, IgG+ and IgA+ B-lymphocytes but their terminal differentiation into plasma cells could not be induced by pokeweed mitogen (PWM) in vitro. On the other hand, the patient's T-cells showed normal helper functions in the PWM system and normal interferon (IFN) production in vitro. However, the IgM+ B-cells can be induced to differentiate into IgM secreting cells by Epstein-Barr virus (EBV), suggesting that the genetic mechanism for synthesis of the component immunoglobulin proteins is present. T-cell functions were impaired, as shown by delayed type cutaneous hypersensitivity (DTH) and mitogen response. The data suggest that the selective IgM deficiency of the patient is due mainly to defects in B-cells at the terminal differentiation stage, but immunological abnormalities are present in both B and T-cell systems. Neutrophil functions examined were normal. MC was treated by intravenous injection of IFN without any side effects; however, no clinical improvement was achieved.
一名患有播散性传染性软疣(MC)的16岁女孩被发现血清IgM水平极低,IgG和IgA水平升高,IgE水平高。她外周血IgM +、IgG +和IgA + B淋巴细胞数量正常,但体外商陆有丝分裂原(PWM)不能诱导它们终末分化为浆细胞。另一方面,患者的T细胞在PWM系统中显示正常的辅助功能,体外干扰素(IFN)产生正常。然而,IgM + B细胞可被爱泼斯坦 - 巴尔病毒(EBV)诱导分化为分泌IgM的细胞,这表明存在合成组成免疫球蛋白的遗传机制。如迟发型皮肤超敏反应(DTH)和有丝分裂原反应所示,T细胞功能受损。数据表明,患者选择性IgM缺乏主要是由于终末分化阶段B细胞缺陷,但B和T细胞系统均存在免疫异常。所检测的中性粒细胞功能正常。通过静脉注射IFN治疗MC,无任何副作用;然而,未取得临床改善。