Katz L J, Hoffman R, Ritchey A K, Dainiak N
Yale J Biol Med. 1981 Mar-Apr;54(2):89-94.
Pure red cell aplasia (PRCA) is a heterogeneous disorder. Immunologic abnormalities have recently been uncovered suggesting that both cell-mediated and humoral immune mechanisms may be of etiological importance in PRCA. Utilizing a technique for the cloning of bone marrow erythroid precursors, we determined the in vitro proliferative capacity of erythroid cells obtained from 21 patients with PRCA. Bone marrow cells from one group of patients produced normal or increased numbers of erythroid colonies while the in vitro proliferative capacity of bone marrow cells from a second group was characterized by subnormal erythroid colony formation. Sera obtained from the former group was frequently associated with demonstrable serum inhibitors of erythropoiesis, while PRCA in the latter group was probably the consequence of intrinsic erythroid stem cell defects or pathologic cellular interactions with nonerythroid regulatory cells. This survey of a relatively large population of patients with PRCA provides evidence for the multiple causative mechanisms that can be operative in the production of PRCA.
纯红细胞再生障碍性贫血(PRCA)是一种异质性疾病。最近发现的免疫异常表明,细胞介导的免疫机制和体液免疫机制在PRCA的病因学中可能都具有重要意义。利用一种克隆骨髓红系前体细胞的技术,我们测定了从21例PRCA患者获得的红系细胞的体外增殖能力。一组患者的骨髓细胞产生的红系集落数量正常或增加,而另一组患者骨髓细胞的体外增殖能力则表现为红系集落形成低于正常水平。从前一组患者获得的血清常与可检测到的红细胞生成血清抑制剂相关,而后一组患者的PRCA可能是内在红系干细胞缺陷或与非红系调节细胞的病理性细胞相互作用的结果。对相对大量PRCA患者的这项调查为PRCA发生过程中可能起作用的多种致病机制提供了证据。