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首页> 外文期刊>Revista Cubana de Hematología, Inmunología y Hemoterapia >Long-term assessment of the partial splenectomy in patients with hereditary spherocytosis
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Long-term assessment of the partial splenectomy in patients with hereditary spherocytosis

机译：遗传性球菌病患者部分脾切除术的长期评估

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The effectiveness of partial splenomegalia (PS) was assessed in 17 patients with hereditary spherocytosis seen in the Hematology and Immunology Institute. Age at diagnosis was of 6.71 ± 5.38 months. All of them had splenomegalia. A patient had vesicular lithiasis before intervention. Criteria to presence of splenomegalia were: transfusion requirements (822.4 %), chronic anemia and splenomegalia (11.7 %) and splenomegalia (5.9 %). Age at moment of PS was of 7.0 ± 2.6 years. Hemoglobin (Hb) was low in the 94.1 % of patients; reticulocytes increased in the 100 % and the total and indirect bilirubin was high in the 76.5 % and the 88.2 %, respectively. Current mean age of patients is 16.24 ± 4.26 years with a course time of 9.24 ± 4.47 years. Postoperative laboratory variables showed a significant increase of Hb (p= 4 × 10-9) and a decrease of reticulocytes (p= 0.003). Trend in time of Hb remained stable in levels achieved after intervention in all patients with more than 10 years of operated on, but not for reticulocytes. Two patients showed a growing of splenic fragment; one was associated with a poor clinical-hematological response. There not sepsis or thromboembolism complications after procedure.
机译：在血液学和免疫学研究所观察到的17名遗传性球囊增多症患者中评估了部分脾肿大（PS）的有效性。诊断时的年龄为6.71±5.38个月。他们所有人都有脾肿大。一名患者在干预前患有水疱结石症。出现脾肿大的标准是：输血需求（822.4％），慢性贫血和脾肿大（11.7％）和脾肿大（5.9％）。 PS发生时的年龄为7.0±2.6岁。 94.1％的患者血红蛋白（Hb）较低;网织红细胞增加了100％，总胆红素和间接胆红素分别占76.5％和88.2％。患者的平均年龄为16.24±4.26岁，病程为9.24±4.47年。术后实验室变量显示Hb显着增加（p = 4×10-9）和网织红细胞减少（p = 0.003）。在所有接受手术治疗超过10年的患者中，Hb的时间趋势在干预后达到的水平保持稳定，但对于网织红细胞并非如此。 2例患者显示脾脏碎片增生。一种与不良的临床血液学反应有关。术后无败血症或血栓栓塞并发症。

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