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原发性自主神经功能衰竭所致贫血及其用重组促红细胞生成素的逆转。

The anemia of primary autonomic failure and its reversal with recombinant erythropoietin.

作者信息

Biaggioni I, Robertson D, Krantz S, Jones M, Haile V

机构信息

Vanderbilt University, Nashville, Tennessee.

出版信息

Ann Intern Med. 1994 Aug 1;121(3):181-6. doi: 10.7326/0003-4819-121-3-199408010-00004.

DOI:10.7326/0003-4819-121-3-199408010-00004
PMID:8017744
Abstract

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 microns 3); serum iron, 16.5 mumol/L (92 micrograms/dL); total iron binding capacity, 43.3 mumol/L (242 micrograms/dL); ferritin, 184 micrograms/L; serum vitamin B12, 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.

摘要

目的

确定慢性交感神经剥夺是否与贫血及促红细胞生成素反应低下有关。

设计

对严重原发性自主神经功能衰竭患者贫血的患病率及特征进行调查。

地点

一家三级教学医院的自主神经功能衰竭转诊服务机构。

患者

84例有症状性直立性低血压的原发性自主神经功能衰竭患者。

干预

用人重组促红细胞生成素进行开放标签试验。

结果

84例患者中有32例存在贫血(38%;95%可信区间,27%至50%)。直立位患者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例血红蛋白水平低于120 g/L患者的平均值如下:血红蛋白108 g/L(范围87至118 g/L);血细胞比容0.33;校正网织红细胞计数0.008;平均红细胞体积89 fL(89立方微米);血清铁16.5 μmol/L(92 μg/dL);总铁结合力43.3 μmol/L(242 μg/dL);铁蛋白184 μg/L;血清维生素B12 410 pmol/L(556 pg/mL);血清叶酸22.7 nmol/L(10 ng/mL)。未发现血清促红细胞生成素与血红蛋白水平之间存在关联。在9例自主神经功能衰竭且血红蛋白水平低于120 g/L的患者中,有7例血清促红细胞生成素水平降至低于缺铁性贫血患者对应的95%可信区间。重组促红细胞生成素治疗使所有接受治疗的患者(n = 5)以相对低剂量(25至50单位/千克体重,皮下注射,每周3次)的平均血红蛋白水平从108 g/L提高到133 g/L。

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说,因为贫血在严重自主神经功能衰竭患者中很常见,且与促红细胞生成素反应减弱有关。

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