Eber S W, Langendörfer C M, Ditzig M, Reinhardt D, Stöhr G, Soldan W, Schröter W, Tchernia G
Department of Pediatrics, Georg-August-University, D-37075 Göttingen.
Ann Hematol. 1999 Nov;78(11):524-8. doi: 10.1007/s002770050550.
Very late sepsis in splenectomized patients with hereditary spherocytosis has been seen rarely up to now; the frequency and the immunodeficiency causing it are largely unknown. Within the past 7 years we have learned of four cases of sepsis or meningitis (three fatal) in adult patients with hereditary spherocytosis who had been splenectomized years earlier. The estimated frequency of very late postsplenectomy infections is 0.69 cases of sepsis or meningitis in 1000 patient-years (0.46 deaths in 1000 patient-years). Pneumococci were proven in two patients. The surviving patient showed low antibody titers against pneumococcal serotypes even after pneumococcal meningitis and subsequent vaccination. There have been several reports of an insufficient response to pneumococcal vaccination in patients with severe infections. We recommend determination of pneumococcal antibody titers after immunization in every splenectomized patient: Nonresponders to vaccination may be at high risk for overwhelming postsplenectomy infection. Our data demonstrate that there is a lifelong risk for severe postsplenectomy infections and therefore the lasting need for immediate antibiotic therapy in any case with sudden onset of high fever.
迄今为止，遗传性球形红细胞增多症脾切除患者发生极晚期败血症的情况极为罕见；其发生率及导致感染的免疫缺陷状况在很大程度上尚不清楚。在过去7年里，我们了解到4例成年遗传性球形红细胞增多症患者在多年前接受脾切除术后发生败血症或脑膜炎（3例死亡）。脾切除术后极晚期感染的估计发生率为每1000患者年0.69例败血症或脑膜炎（每1000患者年0.46例死亡）。两名患者的病原菌被证实为肺炎球菌。即便在发生肺炎球菌脑膜炎及随后接种疫苗后，存活患者针对肺炎球菌血清型的抗体滴度仍较低。已有数篇报道称严重感染患者对肺炎球菌疫苗接种反应不足。我们建议对每例脾切除患者在免疫接种后测定肺炎球菌抗体滴度：对疫苗无反应者可能发生暴发性脾切除术后感染的风险较高。我们的数据表明，脾切除术后存在发生严重感染的终身风险，因此在任何突然高热发作的情况下都始终需要立即进行抗生素治疗。
