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诺尔伯顿型戈谢病的血液学检查结果

Hematological findings in the Norrbottnian type of Gaucher disease.

作者信息

Tibblin E, Dreborg S, Erikson A, Håkansson G, Svennerholm L

出版信息

Eur J Pediatr. 1982 Nov;139(3):187-91. doi: 10.1007/BF01377354.

DOI:10.1007/BF01377354
PMID:7160406
Abstract

A controlled study of hematological changes was performed on 14 patients with the Norrbottnian type of Gaucher disease, seven of whom had been splenectomized at least one year before the study. Each patient had two controls matched to age, sex and habitation. Before splenectomy the patients had slight to moderate microcytic, hypochromic anemia which changed after splenectomy to macrocytic, hypochromic anemia. The splenectomized patients had a high percentage of target cells (mean frequency 33%) and a significantly increased hypo-osmotic resistance of the erythrocytes which showed a positive correlation with the number of target cells. Before removal of the spleen the patients had slight leukopenia with a relative increase of mononuclear cells and moderate thrombocytopenia. In the splenectomized patients the thrombocyte count was normal and there was moderate leukocytosis with a persistent relative increase of lymphocytes.

摘要

对14例诺尔伯特型戈谢病患者进行了血液学变化的对照研究,其中7例患者在研究前至少一年已行脾切除术。每位患者有两名年龄、性别和居住地相匹配的对照者。脾切除术前,患者有轻度至中度的小细胞低色素性贫血,脾切除术后变为大细胞低色素性贫血。行脾切除术的患者靶形细胞百分比很高(平均频率33%),红细胞低渗抵抗力显著增加,且与靶形细胞数量呈正相关。脾切除术前,患者有轻度白细胞减少,单核细胞相对增多,并有中度血小板减少。在脾切除术后的患者中,血小板计数正常,有中度白细胞增多,淋巴细胞持续相对增多。

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The postsplenectomy blood picture.脾切除术后血象。
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