Warren R P, Stembridge A M, Gardner E J
Clin Exp Immunol. 1985 Jun;60(3):525-31.
Genetic susceptibility to certain cancers is recognized as a contributor to malignancy in man and experimental animals. Colorectal adenocarcinoma associated with Gardner syndrome is considered to be a hereditary form of cancer in which family members are at increased risk because they inherit an autosomal dominant gene for adenomas of the colorectum. The adenomas, if untreated, transform into adenocarcinoma. The purpose of the current study was to characterize immune function in patients with Gardner syndrome since reports exist of immune defects in patients with other forms of hereditary cancer. An analysis of the ability of lymphocytes from the patients to be stimulated by the T cell mitogens, phytohaemmaglutinin and concanavalin A, revealed severely depressed responses by peripheral blood mononuclear cells (PBMC) from all of the patients studied. A depressed response by patient PBMC to the B cell mitogen, pokeweed mitogen, also was observed but the extent of depression was not statistically significant. Natural killer (NK) cell activity of the patients was studied to determine if a possible genetic defect in this function is associated with Gardner syndrome. PBMC from half of the patients had marginally depressed NK cell function. An enumeration of patient cells revealed a significantly lower ratio of T4 (helper) to T8 (suppressor) T cells, but normal percentages of rosette forming, 7.2 (Ia) positive and Leu 11 positive (NK) cells.
某些癌症的遗传易感性被认为是人类和实验动物发生恶性肿瘤的一个因素。与加德纳综合征相关的结肠直肠癌被认为是一种遗传性癌症,其家庭成员患癌风险增加,因为他们继承了一个常染色体显性基因,该基因会导致结肠直肠腺瘤。如果不进行治疗,腺瘤会转变为腺癌。由于有报道称其他形式的遗传性癌症患者存在免疫缺陷,因此本研究的目的是对加德纳综合征患者的免疫功能进行特征描述。对患者淋巴细胞受T细胞有丝分裂原(植物血凝素和刀豆球蛋白A)刺激能力的分析显示,所有研究患者的外周血单个核细胞(PBMC)反应严重降低。还观察到患者PBMC对B细胞有丝分裂原(商陆有丝分裂原)的反应降低,但降低程度无统计学意义。对患者的自然杀伤(NK)细胞活性进行了研究,以确定该功能中可能存在的遗传缺陷是否与加德纳综合征有关。一半患者的PBMC的NK细胞功能略有降低。对患者细胞进行计数显示,T4(辅助性)与T8(抑制性)T细胞的比例显著降低,但玫瑰花结形成细胞、7.2(Ia)阳性细胞和Leu 11阳性(NK)细胞的百分比正常。