Murray J L, Perez-Soler R, Bywaters D, Hersh E M
Am J Hematol. 1986 Jan;21(1):57-66. doi: 10.1002/ajh.2830210108.
The purine metabolic enzymes adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), and 5'nucleotidase (5NT) play an important role in normal lymphocyte differentiation. Abnormal levels of one or all of these enzymes have been associated with immunodeficiency diseases and lymphoproliferative disorders. ADA, PNP, and 5NT activity was measured in peripheral blood T cells from 24 patients with Hodgkin disease (HD) (12 in complete remission and 12 with active disease) to determine whether an association existed between enzyme abnormalities and the decreased cellular immune function previously described in this disorder. HD patients had a significantly decreased absolute lymphocyte count (1,618 +/- 1107/mm3; mean +/- SD) compared to controls (2,320 +/- 980; p less than .001). ADA, PNP, and NT activity was assessed in lymphocyte extracts by measuring the conversion of radiolabeled substrates to products over time. ADA activity expressed as mean +/- SEM nanomoles/10(6) lymphocytes/hr was significantly decreased in T cells from HD patients (84.6 +/- 7.5) compared to controls (128 +/- 12.3; p less than 0.025). Likewise, 5NT was significantly decreased in HD patients (12.7 +/- 1.3) compared to controls (24.0 +/- 3.6; p less than .005). There was not a significant difference in PNP activity between both groups. Low 5NT activity was present irrespective of whether patients had active disease (12.1 +/- 1.5) or were in unmaintained complete remission (14.5 +/- 2.4). These findings suggest that biochemical abnormalities may be responsible for or related to the persistent abnormalities in T-cell function noted throughout the clinical course of HD.
嘌呤代谢酶腺苷脱氨酶(ADA)、嘌呤核苷磷酸化酶(PNP)和5'-核苷酸酶(5NT)在正常淋巴细胞分化过程中发挥重要作用。这些酶中一种或全部的异常水平与免疫缺陷疾病和淋巴增殖性疾病有关。检测了24例霍奇金病(HD)患者外周血T细胞中的ADA、PNP和5NT活性(12例完全缓解,12例疾病活动期),以确定酶异常与先前在该疾病中描述的细胞免疫功能下降之间是否存在关联 。与对照组相比,HD患者的绝对淋巴细胞计数显著降低(1,618 +/- 1107/mm3;平均值 +/- 标准差)(2,320 +/- 980;p < 0.001)。通过测量放射性标记底物随时间转化为产物的过程,评估淋巴细胞提取物中的ADA、PNP和NT活性。与对照组(128 +/- 12.3;p < 0.025)相比,HD患者T细胞中以平均 +/- 标准误纳摩尔/10(6)淋巴细胞/小时表示的ADA活性显著降低(84.6 +/- 7.5)。同样,与对照组(24.0 +/- 3.6;p < 0.005)相比,HD患者的5NT显著降低。两组之间PNP活性没有显著差异。无论患者是疾病活动期(12.1 +/- 1.5)还是未维持完全缓解期(14.5 +/- 2.4),均存在低5NT活性。这些发现表明,生化异常可能是HD临床病程中T细胞功能持续异常的原因或与之相关。