遗传性球形红细胞增多症的扫描电镜诊断

The application of scanning electron microscope on diagnosis and study of hereditary spherocytosis

目的：为探讨遗传性球形红细胞增多症异形红细胞的形态分类，发生演变，发病机理和诊断及鉴别，本文运用扫描电镜技术对遗传性球形红细胞增多症的血红细胞进行研究。方法：５例遗传性球形红细胞增多症患者及２例家系成员的静脉血经抗凝和固定，离心和漂洗，干燥和喷金后置于扫描电镜下观察。结果：在观察红细胞从盘形到口形，最终至球形的过程中，作者对各型红细胞直径和中央凹陷直径及细胞厚度的变化作了测量，并结合细胞表面形态和测量结果将病变红细胞分成５个亚型，即：盘口型－碗口型－球口型－口球型－典型球形（包括泪滴状红细胞），其他少量出现的异常红细胞（棘状和靶状红细胞）则归于畸变类，结果表明这对于判断细胞类型和诊断本病很有帮助。

Aim:In order to study the erythrocyte morphorlogical classification and development evolution,pathogenesis,diagnosis and differentiational diagnosis of hereditary spherocytosis (HS),the author made researchs on the red blood cells of HS.Method:After antiagglutination,fixation,centrifugalization,washing,dehydration,dry and gold plating treatment,the blood samples of 5 cases of HS and 2 cases of their family members was observed under S 360 scanning electron microscope.Result:According to the different surface pattern of erythrocytes and diameter of erythrocytes and its surface cave as well as the changes of the thickness of erythrocytes,the erythrocytes of HS were divided into 5 subtypes which were dish like stomatocyte type,bowl like stomatocyte type,ball like stomaocyte type,mouth like spherocyte type and typical spherocyte (include teardrop like type).The other abnormal erythrocytes which were rarely seen such as acanthocyte,target like cell were not classified because they might be malformed cells.Discussion:1.Clinical manifestations:HS is one kind of dominant or recessive hereditary disease.Hemolysis,anemia and splenomegaly are among the symptoms of HS.2.Pathogenesis:Recently,many scientific researchs have demonstrated that HS is mainly caused by spectrin deficiency,ankyrin 1 and band 3 reduction and so on.3.Development evolution of spherocytes:According to the picture under scanning electron microscopy,the author considered that the way of development evolution of spherocytes is as follows:dish like type (normal red blood cell)→dish like stoma tocyte type→bowl like stomatocyte type→ball like stomaocyte type→mouth like spherocyte type→typical spherocyte.The way(dish like type→acnthrocyte type→spherocyte) is uncertain.4.Diagnosis:When the spherocytes of blood exceed in number by 20% (the stomatocytes are not included),the diagnosis of HS should be considered.The erythrocytes classification of HS was very useful to diagnose HS.The disease which needs to be differentitated is hereditary stomatocytosis.5.Treament:Splenectomy is an effective treament for HS.