Elevated red cell adenosine deaminase activity: a marker of disordered erythropoiesis in Diamond‐Blackfan anaemia and other haematologic diseases
Elevated red cell adenosine deaminase activity: a marker of disordered erythropoiesis in Diamond‐Blackfan anaemia and other haematologic diseases
复制标题
红细胞腺苷脱氨酶活性升高：戴蒙德-布莱克凡贫血和其他血液疾病中红细胞生成紊乱的标志
DOI：
--
复制
发表时间：
1988
影响因子：
6.5
通讯作者：
K. Backer
中科院分区：
文献类型：
--
作者：
B. Glader;K. Backer
钻石-黑粉贫血患儿红细胞腺苷脱氨酶(ADA)活性(1.91±0.90U/g Hb)明显高于儿童一过性红细胞减少(0.80±0.16U/g Hb)和正常人(0.61±0.13U/g)。因此，这些数据进一步支持了这种嘌呤代谢酶的测定在诊断儿童纯红细胞再生障碍性贫血的病因中是有用的。然而，有趣的是，在一些患有急性白血病和其他血液疾病的儿童中也可以看到RBC-ADA活性升高。在急性淋巴细胞白血病(ALL)的儿童中，RBC-ADA活性的增加与贫血的程度成正比。然而，白血病人群中RBC-ADA活性的升高与胎儿血红蛋白浓度无关。这些数据表明，RBC-ADA活性升高可能是红系干细胞功能异常的非特异性表现，这种改变不同于胎儿红细胞生成的重新激活。然而，由于几乎所有钻石-布莱克范贫血患者都表现出RBC-ADA活性升高，这种化学变化可能反映了这种疾病中特有的红系分化损害。
Red‐cell adenosine deaminase (ADA) activity in children with Diamond‐Blackfan anaemia is significantly increased (1·91±0·90 U/g Hb) compared to that seen in transient erythroblastopenia of childhood (0·80±0·16 U/g Hb) or normal individuals (0·61±0·13 U/g). These data thus further support that measurement of this purine metabolic enzyme is useful in diagnosing the cause of pure RBC aplasia in children. Of interest, however, elevated RBC‐ADA activity also is seen in some children with acute leukaemia and other haematologic disorders. In children with acute lymphoblastic leukaemia (ALL), the increase in RBC‐ADA activity is proportional to the degree of anaemia. However, the elevated RBC‐ADA activity in this leukaemic population is not related to the fetal haemoglobin concentration. These data suggest increased RBC‐ADA activity may be a non‐specific manifestation of abnormal erythroid stem cell function, an alteration distinct from that seen with reactivation of fetal erythropoiesis. However, since almost all patients with Diamond‐Blackfan anaemia manifest elevated RBC‐ADA activity, this chemical alteration yet may reflect the specific erythroid differentiation lesion in this disorder.