Frequency of very late fatal sepsis after splenectomy for hereditary spherocytosis: impact of insufficient antibody response to pneumococcal infection

Abstract

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.

Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis

“…Yet, it is likely to expose the patient to a lifelong risk for potentially lethal infections. 8,9 Chronic hypercoagulable states leading to thrombosis have also been reported in some human heritable hemolytic disorders and in murine HS. 19 Secondary atherosclerotic events can occur in human subjects after splenectomy for trauma 11 or HS.…”

“…Infectious complications due to circulating encapsulated bacteria can occur irrespective of age and of the time interval after the surgical procedure. 8,9 The risk is particularly high during the first years of life. Although vaccinations and systematic oral penicillin prophylaxis have decreased the overall risk, 10 concerns still exist about serotypes that are not represented in vaccines, penicillin-resistant pneumococcal strains, and lifelong compliance.…”

“…Although vaccinations and systematic oral penicillin prophylaxis have decreased the overall risk, 10 concerns still exist about serotypes that are not represented in vaccines, penicillin-resistant pneumococcal strains, and lifelong compliance. 9 Recent studies have also raised additional concerns regarding long-term complications after splenectomy, such as atherosclerotic events and pulmonary hypertension, [11][12][13] reinforcing the need for alternative treatment strategies. In a previous study, we assessed the potential usefulness of subtotal splenectomy in managing a small population of patients with HS.…”

Incidental Splenic Injury and Splenectomy: An Overview Based on Available Evidence

“…Thirty-nine articles in extensive were discarded for failing to meet the eligibility criteria. Finally, the population in study consisted of 35 articles, 3 of evidence level type 3a (Renzulli et al, 2009;He et al, 2014;Piccolo et al, 2014), 32 of evidence level type 4 (Bagrodia et al, 2014;Barmparas et al, 2015;Bracale et al, 2013;Cassar & Munro, 2009;Coon, 1990;Chung et al, 2011;Davies et al, 2014;Eaton et al, 2000;Eber et al, 1999;Edgren et al, 2014;Flum et al, 2001;Geraci et al, 2014;Gómez Alonso et al, 2001;Ha & Minchin, 2009;Harbrecht et al, 2008;Holubar et al, 2009;Kamath et al, 2009;Malek et al, 2007;Masoomi et al, 2012;McIntyre et al, 2005;Merchea et al, 2012;Nikolaev et al, 1990;Ong et al, 1991;Paredes et al, 2013;Rogers et al, 1980;Singla, et al, 2012;Tan et al, 2011;Urschel, 1993;Wang et al, 2011;Yong et al, 2010; and 1 of evidence level type 5 (American Society for Metabolic & Bariatric Surgery, 2012).…”

“…On the other hand, there are reports of an insufficient response to pneumococcal vaccination in patients with infections (Evidence level type 4) (Eber et al, 1999).…”