Bom-van Noorloos A A, Pegels H G, van Oers R H, Silberbusch J, Feltkamp-Vroom T M, Goudsmit R, Zeijlemaker W P, von dem Borne A E, Melief C J
N Engl J Med. 1980 Apr 24;302(17):933-7. doi: 10.1056/NEJM198004243021702.
Two patients with severe granulocytopenia and recurrent infections of the skin and oropharynx had excess T lymphocytes with receptors for the Fc portion of IgG (T gamma cells) in blood and bone marrow. The abnormal T gamma cells killed antibody-sensitized target cells in vitro (killer-cell activity) but did not suppress immunoglobulin production by B lymphocytes (suppressor-cell activity). T gamma lymphocytes from normal persons showed both killer-cell activity and suppressor-cell activity. In the serum of one patient, granulocyte antibodies, possibly of an autoimmune nature, were detected. The clinical picture in conjunction with the hematologic and immunologic findings characterized the disease of both patients as a distinct entity among the chronic lymphoproliferative diseases of T-cell origin.
两名患有严重粒细胞减少症且皮肤和口咽反复感染的患者,其血液和骨髓中存在带有IgG Fc段受体的过量T淋巴细胞(Tγ细胞)。异常的Tγ细胞在体外可杀死抗体致敏的靶细胞(杀伤细胞活性),但不抑制B淋巴细胞产生免疫球蛋白(抑制细胞活性)。正常人的Tγ淋巴细胞同时具有杀伤细胞活性和抑制细胞活性。在一名患者的血清中检测到可能具有自身免疫性质的粒细胞抗体。结合血液学和免疫学检查结果,两名患者的临床表现将该病特征化为T细胞起源的慢性淋巴细胞增殖性疾病中的一种独特病症。