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本文总结血红蛋白H病73例,并对其临床表现及实验室检查进行分析。血红蛋白H病是广东省成人较为常见的溶血性贫血,散发于本省各地区,与6-磷酸葡萄糖脱氧酶(以下简称G-6-PD)缺乏症的地理分布相重叠。广州市郊某公社本病的发病率超过2/5。本病症状出现的时间受一定因素的影响,本文2例在妊娠及正常哺乳时间内症状明显好转。本病诊断的主要依据是阳性家族史、临床表现及实验室检查。确诊条件是发现阳性血红蛋白H包涵体红细胞及/或血红蛋白H。血红蛋白H包涵体的检查是一种敏感的实验方法,本文全部病例均为阳性。家族调查发现标准型α地中海贫血5例,血红蛋白电泳未出现H血红蛋白,但发现阳性血红蛋白H包涵体红细胞。血红蛋白H包涵体阳性红细胞数目过少时,容易发生漏诊,同时作异丙醇试验有提示和协助诊断的作用。本病容易与缺铁性贫血、肝胆道疾病和继发性贫血相混淆,应注意进行鉴别。患血红蛋白H病时,高铁血红蛋白还原试验不准确,不能作为检查血红蛋白H病患者是否合并G-6-PD缺乏症的实验方法。
This article summarizes 73 cases of hemoglobin H disease, and its clinical manifestations and laboratory tests were analyzed. Hemoglobin H disease is a common haemolytic anemia in adults in Guangdong Province. It is distributed in all regions of the province and overlaps with the geographical distribution of 6-phosphoglucose dehydrogenase (hereinafter referred to as G-6-PD) deficiency. Guangzhou, a commune this disease incidence of more than 2/5. Symptoms of this disease appear to be affected by certain factors, this article 2 cases in pregnancy and normal lactation time significantly improved symptoms. The main basis for diagnosis of this disease is a positive family history, clinical manifestations and laboratory tests. The diagnosis was found to be positive hemoglobin H inclusion and / or hemoglobin H. Hemoglobin H inclusion examination is a sensitive experimental method, all cases are positive in this article. Family survey found that 5 cases of standard α-thalassemia, hemoglobin electrophoresis did not appear H hemoglobin, but found that positive hemoglobin H inclusion of red blood cells. Hemoglobin H inclusion body positive red blood cell number is too small, prone to missed diagnosis, at the same time as isopropanol test tips and assist diagnosis. The disease is easily confused with iron-deficiency anemia, hepatobiliary disease and secondary anemia, should pay attention to identify. Methemoglobin reduction tests are inaccurate when patients have hemoglobin H disease and can not be used as a test to check whether patients with hemoglobin H have a G-6-PD deficiency.