Kuliszkiewicz-Janus Małgorzata, Zimny Anna
Katedra i Klinika Hematologii, Nowotworów Krwi i Transplantacji Szpiku AM we Wrocławiu.
Pol Arch Med Wewn. 2003 Nov;110(5):1327-33.
Glucose-6-phosphate dehydrogenase (G6PD) is one of the most important cytoprotective enzymes for oxidative stress. The WHO classification of G6PD deficiency, based on enzyme activity and clinical significance, distinguishes five variants. Chronic haemolytic process is rare and the main factors causing haemolysis are: infections, substances derived from plants, drugs with high oxidation-reduction potential, stress, ketoacidosis in diabetes and surgery operations. We report two cases of women belonging to the class 3 of the WHO classification in whom haemolysis occured during pregnancy. One of the patients developed two incidents of haemolytic anaemia. The cause of the first episode, nine months before pregnancy, was probably infection of the urinary tract caused by Escherichia coli, but the influence of the drugs also cannot be excluded. Because of the genetic background of this enzymopathy we also examined members of the patients, families but did not find any evidence of G6PD deficiency among them. The reported cases indicate that haemolytic anaemia caused by G6PD deficiency may occur during pregnancy what can lead to many not only haematological but also serious obstetrical complications such as infertility, fetus malformations and even its death. We also draw attention to several difficulties in diagnosing G6PD deficiency especially during haemolysis.
葡萄糖-6-磷酸脱氢酶（G6PD）是最重要的抗氧化应激细胞保护酶之一。世界卫生组织基于酶活性和临床意义对G6PD缺乏症进行分类，区分出五种变体。慢性溶血过程较为罕见，导致溶血的主要因素有：感染、植物源性物质、具有高氧化还原电位的药物、应激、糖尿病酮症酸中毒以及外科手术。我们报告两例属于世界卫生组织分类3级的女性病例，她们在孕期发生了溶血。其中一名患者出现了两次溶血性贫血事件。第一次发作发生在怀孕前九个月，病因可能是大肠杆菌引起的尿路感染，但也不能排除药物的影响。由于这种酶病的遗传背景，我们还对患者的家庭成员进行了检查，但未在他们当中发现任何G6PD缺乏的证据。报告的病例表明，G6PD缺乏引起的溶血性贫血可能在孕期发生，这不仅会导致许多血液学并发症，还会引发严重的产科并发症，如不孕、胎儿畸形甚至胎儿死亡。我们还提请注意诊断G6PD缺乏症存在的几个困难，尤其是在溶血期间。
