Pavón Morán V, Estrada del Cueto M, Fernández Delgado N, Sánchez Damas M, Vergara Rivero R, González Otero A, Vilorio Barreras P, Svarch E
Rev Invest Clin. 2000 May-Jun;52(3):229-33.
To evaluate the hematological and clinical response to partial splenectomy in a group of children with hereditary spherocytosis.
We analyzed the main hematological and clinical features in 13 patients with hereditary spherocytosis submitted to partial splenectomy. The diagnosis of the disease and the hematological studies was made according to standard methods. To establish the inheritance pattern we also studied both parents. During the surgical procedure the upper two thirds of the spleen were removed and the lower pole was preserved. We use prophylactic penicillin (250 mg twice a day) during 3 years.
The diagnosis of the disease was made during the first year of life in 11 patients, 76.9% had neonatal hyperbilirrubinemia, 53.8% showed hepatomegaly and in 69.2% we observed splenomegaly. 84.6% received blood transfusion. The hemoglobin level was significantly increased (p = 0.04) and the reticulocytes significantly diminished (p = 0.01) after splenectomy. No hemolytic crises, blood cell transfusion requirement neither infection was observed after surgery.
Although the number of patients and the post-surgical follow-up is reduced, the hematological recovery observed in our cases suggest that partial splenectomy is a beneficial surgical procedure for the treatment of the typical and severe form of hereditary spherocytosis.
评估一组遗传性球形红细胞增多症患儿行部分脾切除术后的血液学及临床反应。
我们分析了13例行部分脾切除术的遗传性球形红细胞增多症患者的主要血液学及临床特征。疾病诊断及血液学研究均按照标准方法进行。为确定遗传模式，我们还研究了患儿的父母。手术过程中切除脾脏上三分之二，保留脾下极。我们在3年内使用预防性青霉素（每日两次，每次250毫克）。
11例患者在出生后第一年内确诊，76.9%有新生儿高胆红素血症，53.8%有肝肿大，69.2%有脾肿大。84.6%接受过输血。脾切除术后血红蛋白水平显著升高（p = 0.04），网织红细胞显著减少（p = 0.01）。术后未观察到溶血危象、血细胞输血需求及感染。
尽管患者数量及术后随访时间有限，但我们病例中观察到的血液学恢复表明，部分脾切除术对于治疗典型且严重的遗传性球形红细胞增多症是一种有益的手术方式。
