Shih L Y, Leu M L
Department of Internal Medicine, Chang Gung Medical College, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.
Exp Hematol. 1993 Aug;21(9):1239-43.
Five patients with erythrocytosis associated with renal failure on maintenance hemodialysis were investigated for in vitro erythroid progenitor growth and the effect of their uremic sera on normal erythropoiesis. The duration of hemodialysis prior to discovery of erythrocytosis ranged from 1 week to 96 months. None had acquired cystic disease and no other known cause of increased erythropoietin (Epo) production was identified. With the presence of Epo in cultures, all five patients grew erythroid colonies within normal or higher than normal ranges. Three patients formed spontaneous erythroid colonies in the absence of added Epo; all three fulfilled the clinical diagnosis of polycythemia vera (PV). The uremic sera from patients with PV lacked either a stimulating or an inhibiting effect on normal erythropoiesis. The association between renal failure and PV was coincidental. The other two patients without endogenous erythroid colony formation had enhanced erythropoietic activity in their sera, which increasingly stimulated the erythroid colony growth by normal bone marrow cells as the concentration of the uremic serum was increased. The etiology of increased Epo production in these 2 patients remained undefined during long-term follow-up. The present study on five uremic patients with polycythemia showed two different underlying mechanisms of erythrocytosis--characteristic autonomous erythroid proliferation for PV in three patients and inappropriate idiopathic Epo production in two patients.
对5例维持性血液透析的肾衰竭相关红细胞增多症患者进行了体外红系祖细胞生长及尿毒症血清对正常红细胞生成影响的研究。发现红细胞增多症之前的血液透析时间为1周-96个月。所有患者均未患获得性囊性疾病，也未发现其他已知的促红细胞生成素（Epo）产生增加的原因。由于培养中有Epo存在，所有5例患者均在正常或高于正常范围内生长出红系集落。3例患者在未添加Epo的情况下形成了自发性红系集落；这3例均符合真性红细胞增多症（PV）的临床诊断。PV患者的尿毒症血清对正常红细胞生成既无刺激作用也无抑制作用。肾衰竭与PV之间的关联是偶然的。另外2例未形成内源性红系集落的患者血清中的促红细胞生成活性增强，随着尿毒症血清浓度的增加，其对正常骨髓细胞红系集落生长的刺激作用也越来越强。在长期随访中，这2例患者Epo产生增加的病因仍不明确。本项对5例尿毒症性红细胞增多症患者的研究显示，红细胞增多症存在两种不同的潜在机制——3例PV患者具有特征性的自主性红系增殖，2例患者存在不适当的特发性Epo产生。
