Eber S W, Belohradsky B H, Weiss M
Abt. Immunol./Hämotol./Onkol., Univ.-Kinderklinik Zürich, Zurich, Switzerland.
Klin Padiatr. 2001 Sep;213 Suppl 1:A84-7. doi: 10.1055/s-2001-17504.
Asplenia in childhood may be congenital (e.g. Ivemark-syndrome) or acquired (functional hyposplenism in sickle cell disease; after splenectomy or bone marrow transplantation). Hereditary spherocytosis is the most common indication for splenectomy in childhood. Virtually every patient without spleen has a significantly increased risk of severe postsplenectomy infection (mostly caused by Streptococcus pneumoniae). Therefore, vaccinations against pneumococci, haemophilus influenzae and, under certain circumstances, meningococci are recommended. In addition a continuous prophylaxis with antibiotics should be performed for at least three years (or even longer depending on the disease) after splenectomy followed by lifelong interventional application of broad spectrum antibiotics in case of any unclear infection or high fever. This prophylaxis must be started as early as four months of age in sickle cell disease. In future the use of penicillin may be hampered by the growing resistance of pneumococci. Due to this fact the indication for splenectomy in childhood should be restricted to patients with hematologic disease (spherocytosis and other hemolytic anemias, chronic ITP etc.) and moderate to severe symptoms. It is unclear whether partial splenectomy for spherocytosis (and other hemolytic anemias) is an alternative regarding both longlasting reduction of hemolysis and prevention of severe infection. After trauma every effort should be undertaken to preserve a splenic remnant.
儿童无脾症可能是先天性的(如艾维马克综合征)或后天获得性的(镰状细胞病中的功能性脾功能减退;脾切除术后或骨髓移植后)。遗传性球形红细胞增多症是儿童脾切除最常见的指征。实际上,每个无脾患者发生严重脾切除术后感染的风险都显著增加(主要由肺炎链球菌引起)。因此,建议接种肺炎球菌、流感嗜血杆菌疫苗,在某些情况下还应接种脑膜炎球菌疫苗。此外,脾切除术后应连续使用抗生素预防至少三年(或根据病情甚至更长时间),之后如出现任何不明感染或高热,应终身使用广谱抗生素进行干预性治疗。对于镰状细胞病,这种预防措施必须早在四个月大时就开始。未来,肺炎球菌耐药性的不断增加可能会阻碍青霉素的使用。鉴于此,儿童脾切除的指征应仅限于患有血液系统疾病(球形红细胞增多症和其他溶血性贫血、慢性免疫性血小板减少性紫癜等)且有中度至重度症状的患者。对于球形红细胞增多症(和其他溶血性贫血),部分脾切除术在长期减少溶血和预防严重感染方面是否是一种替代方法尚不清楚。外伤后应尽一切努力保留脾脏残余组织。