Takahashi M, Nikkuni K, Tanaka I, Furukawa T, Moriyama Y, Shibata A, Satoh S, Kuwabara Y, Maruyama Y, Matsunaga Y
First Department of Internal Medicine, Niigata University, Japan.
Ann Hematol. 1991 Jul;63(1):9-14. doi: 10.1007/BF01714954.
Inhibitory mechanisms of erythropoiesis in 20 patients with pure red cell aplasia (PRCA) were investigated using the technique of in vitro hematopoiesis and an assay for human parvovirus. Complement-dependent serum inhibitors against late erythroid progenitors (CFU-E) were demonstrated in seven of 19 patients examined, and complement-dependent inhibitors against early erythroid progenitors (BFU-E) were demonstrated in three of these seven patients. Nonspecific and complement-independent inhibitors against CFU-E were thought to be associated with the etiology of PRCA in one patient. Human parvovirus-mediated erythropoietic suppression was demonstrated in a patient with complete remission of acute lymphoblastic leukemia complicated with marrow erythroid aplasia, whose serum showed a perfect inhibition against erythroid progenitor cells. T-cell-mediated erythroid suppression was not demonstrated in the patients examined. These findings reveal that erythroid aplasia is associated with complement-dependent serum erythropoietic inhibitor in some patients (36.8% in the present study) with PRCA, but it is difficult to identify the mechanism of erythroid aplasia in more than half of the patients with PRCA. In addition, our present study discovered the presence of parvovirus-mediated marrow pure red cell aplasia in one adult patient with acute lymphoblastic leukemia.
利用体外造血技术和人细小病毒检测法,对20例纯红细胞再生障碍性贫血(PRCA)患者的红细胞生成抑制机制进行了研究。在19例受检患者中,有7例检测到针对晚期红系祖细胞(CFU-E)的补体依赖性血清抑制剂,在这7例患者中的3例检测到针对早期红系祖细胞(BFU-E)的补体依赖性抑制剂。在1例患者中,认为针对CFU-E的非特异性和非补体依赖性抑制剂与PRCA的病因有关。在1例急性淋巴细胞白血病合并骨髓红系再生障碍且完全缓解的患者中,证实了人细小病毒介导的红细胞生成抑制,其血清对红系祖细胞表现出完全抑制作用。在所检测的患者中未证实T细胞介导的红系抑制。这些发现表明,在一些PRCA患者(本研究中为36.8%)中,红系再生障碍与补体依赖性血清红细胞生成抑制剂有关,但在超过一半的PRCA患者中难以确定红系再生障碍的机制。此外,我们目前的研究在1例成年急性淋巴细胞白血病患者中发现了细小病毒介导的骨髓纯红细胞再生障碍。