De la Concha E G, Garcia-Rodriguez M C, Zabay J M, Laso M T, Alonso F, Bootello A, Fontan G
Clin Exp Immunol. 1982 Sep;49(3):670-6.
Two patients with selective IgM deficiency were studied. Both presented with dermatitis, chronic diarrhoea, recurrent respiratory infections, failure to thrive, elevated serum IgE levels and in vivo impairment of antibody production. No phagocytic or complement abnormalities were found. B lymphocytes with surface IgM were present in normal or high percentage in peripheral blood, and produced normal amounts of IgM in vitro when co-cultured with normal T cells. Patients' T cells did not show excess suppressor function in vitro but had a decreased helper activity for IgM, IgG and IgA production. It is suggested that both patients have an extensive humoral immune deficiency that might be caused by the immunoregulatory T cell defect.
对两名选择性IgM缺乏症患者进行了研究。两人均表现为皮炎、慢性腹泻、反复呼吸道感染、生长发育迟缓、血清IgE水平升高以及体内抗体产生受损。未发现吞噬或补体异常。外周血中表面带有IgM的B淋巴细胞比例正常或偏高,与正常T细胞共培养时在体外产生正常量的IgM。患者的T细胞在体外未表现出过度的抑制功能,但对IgM、IgG和IgA产生的辅助活性降低。提示这两名患者均存在广泛的体液免疫缺陷,可能由免疫调节性T细胞缺陷所致。