Clinical features and management of sepsis in the asplenic patient
- Mark S Pasternack, MD
Mark S Pasternack, MD
- Associate Professor of Pediatrics
- Harvard Medical School
- Section Editors
- Peter F Weller, MD, FACP
Peter F Weller, MD, FACP
- Editor-in-Chief — Infectious Diseases
- Section Editor — Tropical Medicine
- William Bosworth Castle Professor of Medicine
- Harvard Medical School
- Professor of Immunology and Infectious Diseases
- Harvard T. H. Chan School of Public Health
- Morven S Edwards, MD
Morven S Edwards, MD
- Section Editor — Pediatric Infectious Diseases
- Professor of Pediatrics
- Baylor College of Medicine
Splenic function is lost when the spleen has been surgically removed, is congenitally absent, has atrophied following repeated infarction (eg, sickle cell disease), or has infarcted following splenic artery thrombosis (including therapeutic embolization for splenic hemorrhage). In addition, splenic function is reduced in the neonate and may be abnormally reduced (ie, hyposplenism or functional asplenia) when the spleen is engorged with blood (eg, splenic sequestration crisis associated with sickle cell disease, malaria, splenic vein thrombosis) or infiltrated (eg, sarcoidosis, amyloidosis, tumors, or cysts). (See "Approach to the adult patient with splenomegaly and other splenic disorders".)
Asplenic patients and those with impaired splenic function are at risk for a fulminant sepsis syndrome, usually due to Streptococcus pneumoniae. The importance of the spleen for clearance of bacteria and humoral immune response, conditions leading to asplenia, clinical manifestations of infection in the asplenic patient, and the management of postsplenectomy sepsis will be reviewed here. The terms "postsplenectomy sepsis" and "asplenic sepsis" are largely interchangeable, since the functional defects are the same regardless of whether the causative process is congenital or acquired. In this topic review, we will use the term "asplenic sepsis" to include all asplenic patients.
The prevention of sepsis in the asplenic patient is discussed separately. (See "Prevention of sepsis in the asplenic patient".)
ROLE OF THE SPLEEN IN HOST DEFENSE
The sinusoids of the spleen serve as a "fine tooth comb" to filter blood through a series of capillaries as small as 1 micron in diameter to sequester senescent, rigid erythrocytes from the circulation. The mononuclear phagocytes located within this capillary network also ingest circulating bacteria, particularly unopsonized organisms, thus cleansing the roughly 6 percent of cardiac output that perfuses the spleen . The spleen also serves as the largest lymphoid organ within the body and contains nearly half of the body's total immunoglobulin-producing B lymphocytes. Thus, the spleen not only clears bacteria from the circulation but also processes this foreign material to stimulate the production of opsonizing antibody. This function is particularly important in the clearance of encapsulated organisms.
CAUSES OF IMPAIRED SPLENIC FUNCTION
Impaired splenic function can result from splenic disease (hyposplenism) or, more commonly, splenectomy. The causes of splenic disease include:
- Hosea SW, Brown EJ, Hamburger MI, Frank MM. Opsonic requirements for intravascular clearance after splenectomy. N Engl J Med 1981; 304:245.
- Di Sabatino A, Carsetti R, Corazza GR. Post-splenectomy and hyposplenic states. Lancet 2011; 378:86.
- KING H, SHUMACKER HB Jr. Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann Surg 1952; 136:239.
- Rubin LG, Schaffner W. Clinical practice. Care of the asplenic patient. N Engl J Med 2014; 371:349.
- Weitzman S, Aisenberg AC. Fulminant sepsis after the successful treatment of Hodgkin's disease. Am J Med 1977; 62:47.
- Styrt B. Infection associated with asplenia: risks, mechanisms, and prevention. Am J Med 1990; 88:33N.
- Schwartz PE, Sterioff S, Mucha P, et al. Postsplenectomy sepsis and mortality in adults. JAMA 1982; 248:2279.
- Kyaw MH, Holmes EM, Toolis F, et al. Evaluation of severe infection and survival after splenectomy. Am J Med 2006; 119:276.e1.
- Luoto TT, Pakarinen MP, Koivusalo A. Long-term outcomes after pediatric splenectomy. Surgery 2016; 159:1583.
- Theilacker C, Ludewig K, Serr A, et al. Overwhelming Postsplenectomy Infection: A Prospective Multicenter Cohort Study. Clin Infect Dis 2016; 62:871.
- Gopal V, Bisno AL. Fulminant pneumococcal infections in 'normal' asplenic hosts. Arch Intern Med 1977; 137:1526.
- Hifumi T, Fujishima S, Chang B, et al. Fatal overwhelming postsplenectomy infection caused by Streptococcus pneumoniae in mothers within 1 year after delivery: case report. J Infect Chemother 2013; 19:1202.
- Zarrabi MH, Rosner F. Serious infections in adults following splenectomy for trauma. Arch Intern Med 1984; 144:1421.
- Cullingford GL, Watkins DN, Watts AD, Mallon DF. Severe late postsplenectomy infection. Br J Surg 1991; 78:716.
- Eber SW, Langendörfer CM, Ditzig M, et al. Frequency of very late fatal sepsis after splenectomy for hereditary spherocytosis: impact of insufficient antibody response to pneumococcal infection. Ann Hematol 1999; 78:524.
- Holdsworth RJ, Irving AD, Cuschieri A. Postsplenectomy sepsis and its mortality rate: actual versus perceived risks. Br J Surg 1991; 78:1031.
- Thomsen RW, Schoonen WM, Farkas DK, et al. Risk for hospital contact with infection in patients with splenectomy: a population-based cohort study. Ann Intern Med 2009; 151:546.
- Clayer MT, Drew PA, Leong AS, Jamieson GG. IgG-mediated phagocytosis in regenerated splenic tissue. Clin Exp Immunol 1994; 97:242.
- Weissauer W. [Legal requirements of continuing education and control]. Chirurg 1990; 61:suppl 1.
- Ishikawa T, Kubota T, Horigome R, et al. Prevalence of Howell-Jolly bodies caused by partial splenic embolization for portal hypertension. Intern Med 2013; 52:1765.
- Loggie BW, Hinchey EJ. Does splenectomy predispose to meningococcal sepsis? An experimental study and clinical review. J Pediatr Surg 1986; 21:326.
- Martone WJ, Zuehl RW, Minson GE, Scheld WM. Postsplenectomy sepsis with DF-2: report of a case with isolation of the organism from the patient's dog. Ann Intern Med 1980; 93:457.
- Chiappa V, Chang CY, Sellas MI, et al. Case records of the Massachusetts General Hospital. Case 10-2014. A 45-year-old man with a rash. N Engl J Med 2014; 370:1238.
- Shepard CW, Daneshvar MI, Kaiser RM, et al. Bordetella holmesii bacteremia: a newly recognized clinical entity among asplenic patients. Clin Infect Dis 2004; 38:799.
- Rosner F, Zarrabi MH, Benach JL, Habicht GS. Babesiosis in splenectomized adults. Review of 22 reported cases. Am J Med 1984; 76:696.
- Krause PJ, Gewurz BE, Hill D, et al. Persistent and relapsing babesiosis in immunocompromised patients. Clin Infect Dis 2008; 46:370.
- Demar M, Legrand E, Hommel D, et al. Plasmodium falciparum malaria in splenectomized patients: two case reports in French Guiana and a literature review. Am J Trop Med Hyg 2004; 71:290.
- Rabinstein A, Tikhomirov V, Kaluta A, et al. Recurrent and prolonged fever in asplenic patients with human granulocytic ehrlichiosis. QJM 2000; 93:198.
- Han XY, Lin P, Amin HM, Ferrajoli A. Postsplenectomy cytomegaloviral mononucleosis: marked lymphocytosis, TCRgamma gene rearrangements, and impaired IgM response. Am J Clin Pathol 2005; 123:612.
- Xu F, Dai CL, Wu XM, Chu P. Overwhelming postsplenectomy infection due to Mycoplasma pneumoniae in an asplenic cirrhotic patient: case report. BMC Infect Dis 2011; 11:162.
- Sheng CF, Liu BY, Zhang HM, Zheng X. Overwhelming postsplenectomy infection. Genet Mol Res 2015; 14:2702.
- Sawmiller CJ, Dudrick SJ, Hamzi M. Postsplenectomy Capnocytophaga canimorsus sepsis presenting as an acute abdomen. Arch Surg 1998; 133:1362.
- Brigden ML, Pattullo AL. Prevention and management of overwhelming postsplenectomy infection--an update. Crit Care Med 1999; 27:836.
- Lynch AM, Kapila R. Overwhelming postsplenectomy infection. Infect Dis Clin North Am 1996; 10:693.
- Watt KM, Massaro MM, Smith B, et al. Pharmacokinetics of moxifloxacin in an infant with Mycoplasma hominis meningitis. Pediatr Infect Dis J 2012; 31:197.
- ROLE OF THE SPLEEN IN HOST DEFENSE
- CAUSES OF IMPAIRED SPLENIC FUNCTION
- BACTERIAL PATHOGENS
- RISK OF OTHER INFECTIONS
- Parasitic infections
- Cytomegalovirus infections
- Other causes
- CLINICAL MANIFESTATIONS
- Physical examination
- Laboratory findings
- Empiric antibiotics
- - Adults
- Beta-lactam allergy
- - Children
- Adjuvant dexamethasone for meningitis
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS
- Clinical features