ELEVATED RED-CELL ADENOSINE-DEAMINASE ACTIVITY - A MARKER OF DISORDERED ERYTHROPOIESIS IN DIAMOND-BLACKFAN ANEMIA AND OTHER HEMATOLOGIC DISEASES
ELEVATED RED-CELL ADENOSINE-DEAMINASE ACTIVITY - A MARKER OF DISORDERED ERYTHROPOIESIS IN DIAMOND-BLACKFAN ANEMIA AND OTHER HEMATOLOGIC DISEASES
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DOI：
10.1111/j.1365-2141.1988.tb06184.x
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发表时间：
1988-02-01
影响因子：
6.5
通讯作者：
BACKER, K
中科院分区：
文献类型：
--
作者：
GLADER, BE;BACKER, K
患有Diamond-Blackfan贫血的儿童红细胞腺苷脱氨酶（ADA）活性显著增加（1.91 ± 0.01）。0.90 U/g Hb）与在儿童期短暂性成红细胞减少症中所见的（0.80 ± 0.10 U/g Hb）相比。0.16 U/g Hb）或正常个体（0.61 . ±. 0.13 U/g）。因此，这些数据进一步支持，这种嘌呤代谢酶的测量是有用的，在诊断儿童纯红细胞再生障碍性贫血的原因。然而，值得注意的是，在一些患有急性白血病和其他血液病的儿童中也观察到RBC-ADA活性升高。在急性淋巴细胞白血病（ALL）儿童中，RBC-ADA活性的增加与贫血程度成正比。然而，该白血病人群中RBC-ADA活性升高与胎儿血红蛋白浓度无关。这些数据表明RBC-ADA活性增加可能是异常红系干细胞功能的非特异性表现，这种改变与胎儿红细胞生成重新激活不同。然而，由于几乎所有的Diamond-Blackfan贫血患者都表现出RBC-ADAD活性升高，这种化学变化可能反映了这种疾病中特定的红细胞分化病变。
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-ADAD activity, this chemical alteration yet may reflect the specific erythroid differentiation lesion in this disorder.