【作者】 王旭；

【导师】 李薇；

【作者基本信息】 吉林大学 ， 临床医学， 2007， 硕士

【摘要】 低增生性骨髓增生异常综合征(HMDS)与再生障碍性贫血(AA)在临床表现和实验室诊断上有许多重叠之处,如两者均有全血细胞减少,骨髓增生减低,有的AA早期或治疗后可出现病态造血等,此外亦有文献报道两者具有相似的病理生理机制,因此围绕HMDS与AA存在许多的争议,已引起了医学界的高度重视,为了进一步提高对HMDS及AA的认识,本文从一般资料,骨髓检查,外周血细胞计数及涂片检查,免疫学及疾病转型五个方面就我院2000年12月至2006年11月确诊为HMDS的患者25例与同期住院(随机抽取)的确诊为SAA的患者25例,确诊为CAA的患者25例分别进行回顾性对比分析,以期总结HMDS与SAA及CAA临床资料的异同点。得出如下结论:(1)HMDS组平均年龄大于SAA组,SAA组较HMDS组更易出现出血。HMDS组与CAA组在上述指标的比较上无显著性差异。(2)HMDS组白细胞计数、中性粒细胞计数、中性粒细胞比例及血小板值高于SAA组,淋巴细胞比例低于SAA组。HMDS与CAA组在上述指标的比较上无显著性差异。(3)骨髓原始细胞的有无及比例大小对于HMDS与SAA及CAA的鉴别有重要意义。(4)绝大多数的HMDS组患者骨髓及外周血存在病态造血,且骨髓涂片常表现为两系或三系的病态造血,而仅有少数的SAA组及CAA组患者骨髓及外周血存在病态造血,主要表现为单一的红系病态造血,相对于SAA组及CAA组,病态巨核细胞是HMDS特有的。(5)HMDS组骨髓造血面积、早幼粒、中幼粒、早幼红、中幼红比例及每片巨核细胞值均高于CAA组及SAA组,骨髓淋巴细胞比例低于CAA组及SAA组,此外骨髓晚幼粒及晚幼红细胞比例高于SAA组,上述指标有助于HMDS与SAA及CAA的鉴别。更多还原

【Abstract】 Hypoplasia myelodysplastic syndrome(HMDS) is a special type of myelodysplastic syndrome, which is characterized by the hypocellular bone marrow cells and dyspoiesis. it is difficult to distinguish hypoplasia myelodysplastic syndrome from aplastic anemia (AA)clinically,and has given rise to the medical dispute very seriously in recent years.Objective:To improve the comprehension of hypoplasia myelodysplastic syndrome and aplastic anemia , summarize the difference of clinical data between hypoplasia myelodysplastic syndrome and aplastic anemia.Method:Retrospective analysis of clinical information from five aspects which include general information, bone marrow examination , CBC and peripheral blood smears examination, immunology and diease transconformation。There are 25 patients with HMDS and 50 patients with AA (25 patients with SAA,25 patients with CAA) who came to our hospital from December , 2000 to November ,2006 are in my investigation.The specimen of CBC and autoantibody examination is venous blood and that of peripheral blood cells morphology examination is peripheral blood. The spots of specimen collection of BM cytology examination and BM patho-examination are followed: anterior superior iliac spine, posterior superior iliac spine or breast bone. BM examinations with different spots are executed at least two times. bone marrow smears and biopsies for routine inspection , iron staining and chromosome examination are also completed .In the aspect of statistics, measurement data is presented in mean±standard deviation ; measurement information between the two groups using t-test or the Wilcoxon rank sum test analysis; Qualitative information is presented in frequency (rate), the difference between the groups using X2 test or Fisher’s Exact Test. All the calculation is completed by SAS software , P<0.05 was significant difference. Results : (1) General information : The average age of SAA group was lower than HMDS group,and was proner to bleeding.(2) Bone marrow examination①The bone marrow haematogenesis area of HMDS group is larger than SAA group and CAA group , the ratio of myeloblast , premyelocyte, myelocyte,metagranulocyt,proerythroblast,basophilic normoblas,polychromatic erythroblast , eosinophilic erythroblast , megakaryocyte values of per unit in bone marrow of HMDS goup were higher than SAA group. the ratio of myeloblast ,premyelocyte, myelocyte,proerythroblast,basophilic normoblas, polychromatic erythroblast, megakaryocyte values of per unit in bone marrow of HMDS goup were higher than CAA group.The lymphocytes ratio in bone marrow of HMDS group was lower than SAA group and CAA group.②96% cases of HMDS group had dyspoiesis in bone marrow smear ,which are mainly dyserythropoiesis together with dysgranulopoiesis or/and dysmegakar- yocytopoiesis,only 28% cases of SAA and CAA group had dyspoiesis, which are mainly dyserythropoiesis. dysmegakaryocytopoiesis is unique to the HMDS groupcompared to SAA group and CAA group.③15 cases of patients in HMDS group , 3 cases of patients in SAA group and 7 cases of patients in CAA accepted bone marrow iron staining exmination,iron outside or within cell of HMDS Group and SAA group and CAA group were almost increased.④12 patients in HMDS group accepted bone marrow chromosome exmination , the result were 1 patient with Abnormal karyotype( 47,XY,+7〔10〕) ,3 patients with normal karyotype , 8 patients without result , 1 patients in SAA group accepted bone marrow chromosome exmination , without result, 2 patients in CAA accepted bone marrow chromosome exmination,all the results were normal.(3)CBC and peripheral blood smears examination:①CBC, leukocyte count, neutrophil count, neutrophil ratio and platelet value of SAA group were lower than HMDS group. Lymphocytes ratio was higher than HMDS group There were no difference of the index above between HMDS group and CAA group②T here were 2 cases of HMDS group had original cells in peripheral blood,which were not seen in SAA group and CAA group。64% cases of HMDS group had dyspoiesis in peripheral blood,only 12% cases of SAA and 8%cases of CAA group had dyspoiesis.(4)Immunology:there were no patients had Autoimmune disease in three groups. All the groups had patients whose serum contained autoantibody.(5)Disease transformation : there were no patients had disease transformation in three groups.Conclusion:(1) The average age of SAA group was lower than HMDS group , SAA group was proner to bleeding. (2) leukocyte count, neutrophil count, neutrophil ratio and platelet value of SAA group were lower than HMDS group. Lymphocytes ratio was higher than HMDS group. There were no difference of the index above between HMDS group and CAA group.(3) blast cell had significance in distinguish HMDS group from AA. group (4) most cases of HMDS group had dyspoiesis in bone marrow smear ,which are mainly dyserythropoiesis together with dysgranulopoiesis or/and dysmegakaryoc- ytopoiesis,only minority cases of SAA and CAA group had dyspoiesis, which were mainly dyserythropoiesis. dysmegakaryocytopoiesis was unique to the HMDS group compared to SAA group and CAA group. (5) The bone marrow haematogenesis area of HMDS group is larger than SAA group and CAA group , the ratio of myeloblast , premyelocyte, myelocyte, metagranulocyt, proerythroblast,basophilic normoblas,polychromatic erythroblast , eosinophilic erythroblast , megakaryocyte values of per unit in bone marrow of HMDS goup were higher than SAA group. the ratio of myeloblast, premyelocyte, myelocyte,proerythroblast,basophilic normoblas, polychromatic erythroblast, megakaryocyte values of per unit in bone marrow of HMDS goup were higher than CAA group.The lymphocytes ratio in bone marrow of HMDS group was lower than SAA group and CAA group.更多还原