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遗传性球形红细胞增多症,是一种遗传性慢性溶血性贫血。临床上常有不同程度贫血,黄疸和脾肿大。它是一种常染色体显性遗传性疾病,引起红细胞遗传性内在缺陷,使红细胞变小而且厚,接近于球形,一般认为本病是由于红细胞膜结构先天异常所致,使钠离子容易进入红细胞内,结果红细胞内渗透压增高,大量水份进入红细胞以致使红细胞变成球形。红细胞内钠离子增高,需消耗更多的ATP酶,以将钠离子泵出。由于红细胞的钠泵功能亢进,ATP的代偿性亢进,能量供给不足,使红细胞过早的衰老,球形化,同时由于球形红小而且厚,“可塑性”减低,使它不易通过脾随,故在脾内滞留下来,由于脾内氧分压及PH都较低,血流缓慢,其酶能量代谢障碍,球形红细胞又缺乏变形能力,在脾脏步球形化,脆性更加增高,因而易于在微循环中受到挤压和撞击碎裂,也容易被巨噬细胞吞噬,这种红细胞寿命仅在15天左右。 现将我院在1975年——1978年收治10例球形红细胞增多症实验室检查结果列表如下页。
Hereditary spherocytosis, is a hereditary chronic hemolytic anemia. Often varying degrees of clinical anemia, jaundice and splenomegaly. It is an autosomal dominant genetic disease, causing hereditary genetic defects of red blood cells, so that red blood cells smaller and thicker, close to the ball, the disease is generally believed that the red cell membrane structure is due to anomalies caused by sodium ions easily enter the red blood cells Within the results of increased intracellular pressure within the red blood cells, a large amount of water into the red blood cells so that the red blood cells into a spherical. Red blood cells increased sodium ions, need to consume more ATPase, in order to pump out sodium ions. As erythrocyte sodium pump function hyperthyroidism, ATP compensatory hyperthyroidism, lack of energy supply, premature aging of red blood cells, spheroidization, at the same time as a small red ball and thick, “plasticity” to reduce, it is not easy to pass through the spleen, so Stranded in the spleen, due to the partial pressure of oxygen in the spleen and PH are lower, blood flow is slow, the enzyme energy metabolism disorder, spherical erythrocytes and lack of deformation capacity in the spleen, ball-shaped, more brittle increased, and thus easy to microcirculation By squeezing and impact fragmentation, but also easily swallowed by macrophages, the life span of red blood cells in only 15 days or so. Now in our hospital in 1975 - 1978, 10 cases of polycythemia punctatus laboratory test results are listed on the following page.