THE ANEMIA OF PRIMARY AUTONOMIC FAILURE AND ITS REVERSAL WITH RECOMBINANT ERYTHROPOIETIN
THE ANEMIA OF PRIMARY AUTONOMIC FAILURE AND ITS REVERSAL WITH RECOMBINANT ERYTHROPOIETIN
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DOI：
10.7326/0003-4819-121-3-199408010-00004
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发表时间：
1994-08-01
影响因子：
39.2
通讯作者：
HAILE, V
中科院分区：
文献类型：
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作者：
BIAGGIONI, I;ROBERTSON, D;HAILE, V
目的：确定慢性交感神经剥夺是否与贫血和低促红细胞生成素反应有关。设计：调查严重自主神经衰竭患者的贫血患病率和特征。背景：在一所三级教学医院提供自主神经衰竭转诊服务。患者：84例有症状的直立性低血压的原发性自主神经衰竭患者。干预：用重组人红细胞生成素进行开放式试验。结果：84例患者中有32例贫血(38%；95%CI，27%~50%)。直立患者的血浆去甲肾上腺素水平在血红蛋白水平较低的患者组中较低。22例血红蛋白<120g/L患者的平均值如下：血红蛋白108g/L(范围87~118g/L)；红细胞压积0.33；校正网织红细胞计数0.008；平均红细胞体积89fl(89mU cm(3))；血清铁16.5mU/L(92mU g/dL)；总铁结合量43.3mU/L(242mU/dL)；铁蛋白184mU/L；血清维生素B-12 410pmol/L(556pg/ml)；血清叶酸22.7nmol/L(10 ng/mL)。未发现血清促红细胞生成素与血液中血红蛋白水平之间的关系。在9名血红蛋白水平低于120g/L的自主神经衰竭患者中，有7名患者的血清促红细胞生成素水平降至与缺铁性贫血患者相对应的95%可信区间以下。重组促红细胞生成素治疗改善了所有患者(n=5)的平均血红蛋白水平(从108克/L到133克/公斤)，治疗剂量相对较低(25-50单位/公斤体重，每周三次)。结论：我们的数据支持交感神经系统刺激人类红细胞生成的假设，因为贫血在严重自主神经衰竭的患者中经常发生，并与红细胞生成素反应迟钝有关。
Objective: To determine if chronic sympathetic deprivation is associated with anemia and a low erythropoietin response.Design: Survey of the prevalence and characteristics of anemia in patients with severe primary autonomic failure.Setting: A referral service for autonomic failure in a tertiary teaching hospital.Patients: 84 patients with primary autonomic failure who had symptomatic orthostatic hypotension.Intervention: Open-label trial with human recombinant erythropoietin.Results: Anemia was present in 32 of 84 patients (38%; 95% Cl, 27% to 50%). Plasma norepinephrine levels, measured in patients standing upright, were lower in the patient group with lower hemoglobin levels. Mean values in 22 patients with a hemoglobin level of less than 120 g/L were as follows: hemoglobin, 108 g/L (range, 87 to 118 g/L); hematocrit, 0.33; corrected reticulocyte counts, 0.008; mean corpuscular volume, 89 fL (89 mu cm(3)); serum iron, 16.5 mu mol/L (92 mu g/dL); total iron binding capacity, 43.3 mu mol/L (242 mu g/dL); ferritin, 184 mu g/L; serum vitamin B-12, 410 pmol/L (556 pg/mL); and serum folate, 22.7 nmol/L (10 ng/mL). No relation was found between serum erythropoietin and blood hemoglobin levels. In seven of nine patients with autonomic failure who had hemoglobin levels less than 120 g/L, serum erythropoietin levels decreased below the 95% confidence interval corresponding to patients with iron deficiency anemia. Therapy with recombinant erythropoietin improved mean hemoglobin levels (from 108 to 133 g/L) in all patients treated (n = 5) at relatively low doses (25 to 50 units/kg body weight, subcutaneously, three times a week).Conclusions: Our data support the hypothesis that the sympathetic nervous system stimulates erythropoiesis in humans because anemia is a frequent occurrence in patients with severe autonomic failure and is associated with a blunted erythropoietin response.