Overwhelming post-splenectomy infection

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Overwhelming post-splenectomy infection
Other namesOverwhelming post-splenectomy sepsis (OPSS) [1]
Specialty Infectious disease
Usual onset24–48 hours following presentation with mild viral symptoms [2]
CausesExposure to pathogens following splenectomy or asplenia
Risk factors Splenectomy in the past 2–3 years, removal of spleen for hematological reasons, being under the age of 2 [2]
Prognosis Almost invariably fatal without treatment [2]

An overwhelming post-splenectomy infection (OPSI) is a rare but rapidly fatal infection occurring in individuals following removal (or permanent dysfunction) of the spleen. The infections are typically characterized by either meningitis or sepsis, and are caused by encapsulated organisms including Streptococcus pneumoniae . [3] It is a medical emergency and requires immediate treatment. Death has been reported to occur within 12 hours. [4]

Contents

The spleen is necessary for protection against encapsulated bacteria (see Mechanism) and as such when removed by splenectomy it can lead to rapid unchallenged infection by encapsulated bacteria. The rapid progression from mild viral symptoms to sepsis is one of the things that makes OPSI particularly dangerous.

Another source of infection [5] are species of Babesia , which are tick-borne parasites that cause babesiosis. [6]

Signs and symptoms

OPSI may initially present with mild viral symptoms such as fever or coughing, however later in infection symptoms may include shakes, shivers, chills, diarrhea, vomiting, malaise, myalgia, headache and abdominal pain. [2] [4]

The disease progresses rapidly from the above mentioned symptoms to coma to refractory septic shock and finally death in as little as 24 hours. [4]

Mechanism

The spleen contains many macrophages (part of the reticuloendothelial system), which are immune cells that phagocytose (eat) and destroy bacteria. In particular, these macrophages are activated when bacteria are bound by IgG antibodies (IgG1 or IgG3) or the complement component C3b. These types of antibodies and complement are immune substances called opsonizers, molecules that bind to the surface of bacteria to facilitate phagocytosis.

When the spleen is no longer present (asplenia), IgG and C3b are still bound to bacteria, but they cannot be removed from the blood circulation due to the loss of the splenic macrophages. Hence the bacteria are free to cause infection.

Patients without a spleen often need immunizations against pathogens that normally require opsonization and phagocytosis by macrophages in the spleen. These include common human pathogens with bacterial capsules ( Streptococcus pneumoniae, Salmonella typhi, Neisseria meningitidis, E. coli, Hemophilus influenzae, Streptococcus agalactiae, Klebsiella pneumoniae, Pseudomonas aeruginosa ). Capsules made of polysaccharides (sugars) permit bacteria to evade phagocytosis by macrophages alone, since only proteins are directly recognized by macrophages in phagocytosis. So humoral immunity in forms of IgG and complement proteins is the human immune system's response against bacterial capsules.

Prevention

Measures to prevent OPSI include vaccination, prophylactic antibiotics and patient education. [7] [8] [9]

Patient education

Knowledge of the risks of asplenia correlates with a greatly reduced risk of OPSI, thus patient education is vital to preventing OPSI and may be the most important factor for preventing OPSI. [10] More and more people are increasingly getting their healthcare information from the internet and the lack of reliable, readable and comprehensive information on the risks of asplenia and splenectomy poses a preventable risk factor for asplenic individuals. [10] The majority (as many as 84%) of asplenic individuals are unaware of the risks of asplenia. [4] Encouraging the wearing of bracelets with information about the condition, the carrying of antibiotics, seeking medical advice before travel, especially to places where malaria and babesia is endemic and seeking immediate medical attention following a bite from an animal has been shown to reduce OPSI risk. [4] [2]

Vaccination

The Centers for Disease Control and Prevention's annual vaccine recommendations includes specifics for individuals without a functioning spleen. [11]

The Green Book (immunisation guidance, UK) in chapter 7 [12] covers immunisation of people with underlying medical conditions that affect immunity which includes asplenic patients.

As there are a range of different pneumococcal vaccines, the patient should be offered the most up to date ones (typically 23 valent polysaccharide vaccine and 13 valent conjugate vaccine), if they have not had them already as part of standard schedule. Repeat doses are recommended in patients without a spleen.

The CDC recommends against live vaccines and has specific advice for travellers, which includes malaria avoidance for asplenic individuals. [13]

Prognosis

OPSI is almost always fatal without treatment, but modern treatment has decreased the mortality to approximately 40–70 percent. [2] [3] [14] Individuals with OPSI are most commonly treated with antibiotics and supportive care. [7]

Epidemiology

The risk of OPSI is 0.23–0.42 percent per year, with a lifetime risk of 5 percent. [7] Most infections occur in the first few years following splenectomy, but the risk of OPSI is lifelong. [3] [15]

The risk is greatest for children and elderly (70+ years old), but it can happen at any age. Greater risk is associated with splenectomy for hematological conditions such as sickle cell anemia, thalassemia and tumours when compared to splenectomy due to trauma. [2] [4]

Related Research Articles

<i>Streptococcus</i> Genus of bacteria

Streptococcus is a genus of gram-positive coccus or spherical bacteria that belongs to the family Streptococcaceae, within the order Lactobacillales, in the phylum Bacillota. Cell division in streptococci occurs along a single axis, so as they grow, they tend to form pairs or chains that may appear bent or twisted. This differs from staphylococci, which divide along multiple axes, thereby generating irregular, grape-like clusters of cells. Most streptococci are oxidase-negative and catalase-negative, and many are facultative anaerobes.

<span class="mw-page-title-main">Spleen</span> Organ recycling old red blood cells and also housing lymphocytes

The spleen is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes from Ancient Greek σπλήν (splḗn).

<span class="mw-page-title-main">Waterhouse–Friderichsen syndrome</span> Medical condition

Waterhouse–Friderichsen syndrome (WFS) is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. Typically, it is caused by Neisseria meningitidis.

<span class="mw-page-title-main">Spherocytosis</span> Medical condition

Spherocytosis is the presence of spherocytes in the blood, i.e. erythrocytes that are sphere-shaped rather than bi-concave disk shaped as normal. Spherocytes are found in all hemolytic anemias to some degree. Hereditary spherocytosis and autoimmune hemolytic anemia are characterized by having only spherocytes.

Bloodstream infections (BSIs) are infections of blood caused by blood-borne pathogens. The detection of microbes in the blood is always abnormal. A bloodstream infection is different from sepsis, which is characterized by severe inflammatory or immune responses of the host organism to pathogens.

<span class="mw-page-title-main">Splenectomy</span> Surgical removal of the spleen

A splenectomy is the surgical procedure that partially or completely removes the spleen. The spleen is an important organ in regard to immunological function due to its ability to efficiently destroy encapsulated bacteria. Therefore, removal of the spleen runs the risk of overwhelming post-splenectomy infection, a medical emergency and rapidly fatal disease caused by the inability of the body's immune system to properly fight infection following splenectomy or asplenia.

<span class="mw-page-title-main">Hereditary spherocytosis</span> Medical condition

Hereditary spherocytosis (HS) is a congenital hemolytic disorder wherein a genetic mutation coding for a structural membrane protein phenotype causes the red blood cells to be sphere-shaped (spherocytosis), rather than the normal biconcave disk shape. This abnormal shape interferes with the cells' ability to flex during blood circulation, and also makes them more prone to rupture under osmotic stress, mechanical stress, or both. Cells with the dysfunctional proteins are degraded in the spleen, which leads to a shortage of erythrocytes and results in hemolytic anemia.

<i>Streptococcus pneumoniae</i> Species of bacterium

Streptococcus pneumoniae, or pneumococcus, is a Gram-positive, spherical bacteria, alpha-hemolytic member of the genus Streptococcus. They are usually found in pairs (diplococci) and do not form spores and are non motile. As a significant human pathogenic bacterium S. pneumoniae was recognized as a major cause of pneumonia in the late 19th century, and is the subject of many humoral immunity studies.

Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism.

<span class="mw-page-title-main">Bacterial pneumonia</span> Disease of the lungs

Bacterial pneumonia is a type of pneumonia caused by bacterial infection.

<span class="mw-page-title-main">Splenomegaly</span> Enlargement of the spleen

Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload, which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusae, is an important sign of portal hypertension.

An autosplenectomy is a negative outcome of disease and occurs when a disease damages the spleen to such an extent that it becomes shrunken and non-functional. The spleen is an important immunological organ that acts as a filter for red blood cells, triggers phagocytosis of invaders, and mounts an immunological response when necessary. Lack of a spleen, called asplenia, can occur by autosplenectomy or the surgical counterpart, splenectomy. Asplenia can increase susceptibility to infection. Autosplenectomy can occur in cases of sickle-cell disease where the misshapen cells block blood flow to the spleen, causing scarring and eventual atrophy of the organ. Autosplenectomy is a rare condition that is linked to certain diseases but is not a common occurrence. It is also seen in systemic lupus erythematosus (SLE).

<span class="mw-page-title-main">Pneumococcal polysaccharide vaccine</span> Pneumonia vaccine

Pneumococcal polysaccharide vaccine, sold under the brand name Pneumovax 23, is a pneumococcal vaccine that is used for the prevention of pneumococcal disease caused by the 23 serotypes of Streptococcus pneumoniae contained in the vaccine as capsular polysaccharides. It is given by intramuscular or subcutaneous injection.

<i>Neisseria meningitidis</i> Species of bacterium that can cause meningitis

Neisseria meningitidis, often referred to as the meningococcus, is a Gram-negative bacterium that can cause meningitis and other forms of meningococcal disease such as meningococcemia, a life-threatening sepsis. The bacterium is referred to as a coccus because it is round, and more specifically a diplococcus because of its tendency to form pairs.

Community-acquired pneumonia (CAP) refers to pneumonia contracted by a person outside of the healthcare system. In contrast, hospital-acquired pneumonia (HAP) is seen in patients who have recently visited a hospital or who live in long-term care facilities. CAP is common, affecting people of all ages, and its symptoms occur as a result of oxygen-absorbing areas of the lung (alveoli) filling with fluid. This inhibits lung function, causing dyspnea, fever, chest pains and cough.

<span class="mw-page-title-main">Marginal zone</span> Part of the spleen

The marginal zone is the region at the interface between the non-lymphoid red pulp and the lymphoid white-pulp of the spleen.

Capnocytophaga canimorsus is a fastidious, slow-growing, Gram-negative rod of the genus Capnocytophaga. It is a commensal bacterium in the normal gingival flora of canine and feline species, but can cause illness in humans. Transmission may occur through bites, licks, or even close proximity with animals. C. canimorsus generally has low virulence in healthy individuals, but has been observed to cause severe, even grave, illness in persons with pre-existing conditions. The pathogenesis of C. canimorsus is still largely unknown, but increased clinical diagnoses have fostered an interest in the bacillus. Treatment with antibiotics is effective in most cases, but the most important yet basic diagnostic tool available to clinicians remains the knowledge of recent exposure to canines or felines.

<span class="mw-page-title-main">Pneumococcal vaccine</span> Vaccine to prevent infection by the bacteria Stretococcus pneumoniae

Pneumococcal vaccines are vaccines against the bacterium Streptococcus pneumoniae. Their use can prevent some cases of pneumonia, meningitis, and sepsis. There are two types of pneumococcal vaccines: conjugate vaccines and polysaccharide vaccines. They are given by injection either into a muscle or just under the skin.

Pneumococcal infection is an infection caused by the bacterium Streptococcus pneumoniae.

Isolated congenital asplenia is a rare disease in humans that can cause life-threatening bacterial infections in children due to primary immunodeficiency. The infections can include pneumococal sepsis and meningitis.

References

  1. ↑ surgical recall, seventh edition, Lorne H. Blackbourne, page 469.
  2. 1 2 3 4 5 6 7 Luu, Sarah; Spelman, Denis; Woolley, Ian J. (2019). "Post-splenectomy sepsis: preventative strategies, challenges, and solutions". Infection and Drug Resistance. 12: 2839–2851. doi: 10.2147/IDR.S179902 . ISSN   1178-6973. PMC   6748314 . PMID   31571940.
  3. 1 2 3 Waghorn DJ (March 2001). "Overwhelming infection in asplenic patients: current best practice preventive measures are not being followed". Journal of Clinical Pathology. 54 (3): 214–8. doi:10.1136/jcp.54.3.214. PMC   1731383 . PMID   11253134.
  4. 1 2 3 4 5 6 Taniguchi, Leandro Utino; Correia, Mário Diego Teles; Zampieri, Fernando Godinho (December 2014). "Overwhelming post-splenectomy infection: narrative review of the literature". Surgical Infections. 15 (6): 686–693. doi:10.1089/sur.2013.051. ISSN   1557-8674. PMID   25318011.
  5. ↑ Rosner, F. (April 1984). "Babesiosis in splenectomized adults. Review of 22 reported cases". American Journal of Medicine. 76 (4): 696–701. doi:10.1016/0002-9343(84)90298-5. PMID   6424470.
  6. ↑ Centers for Disease Control and Prevention. "About Babesiosis". CDC Parasites. Retrieved 19 June 2018.
  7. 1 2 3 Davidson RN, Wall RA (December 2001). "Prevention and management of infections in patients without a spleen". Clinical Microbiology and Infection. 7 (12): 657–60. doi: 10.1046/j.1198-743x.2001.00355.x . PMID   11843905.
  8. ↑ Working Party of the British Committee for Standards in Haematology Clinical Haematology Task Force (1996). "Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. Working Party of the British Committee for Standards in Haematology Clinical Haematology Task Force". BMJ. 312 (7028): 430–4. doi:10.1136/bmj.312.7028.430. PMC   2350106 . PMID   8601117.
  9. ↑ Davies JM, et al. (2001-06-02). "The prevention and treatment of infection in patients with an absent or dysfunctional spleen - British Committee for Standards in Haematology Guideline up-date". BMJ. 312 (7028): 430–4. doi:10.1136/bmj.312.7028.430. PMC   2350106 . PMID   8601117.
  10. 1 2 Downing, Mark A.; Omar, Ahmed H.; Sabri, Elham; McCarthy, Anne E. (August 2011). "Information on the Internet for asplenic patients: a systematic review". Canadian Journal of Surgery. 54 (4): 232–236. doi:10.1503/cjs.005510. PMC   3191896 . PMID   21651833.
  11. ↑ "2018 Adult Schedule by Health Conditions in Easy-to-read Format for Patients". Centers for Disease Control and Prevention. Retrieved 19 June 2018.
  12. ↑ "Immunisation of individuals with underlying medical conditions: the green book, chapter 7" . Retrieved 2023-08-24.
  13. ↑ Camille Nelson Kotton; Andrew T. Kroger; David O. Freedman. "Advising Travelers with Specific Needs". Travelers' Health. Retrieved 25 October 2018.
  14. ↑ Schwartz PE, Sterioff S, Mucha P, Melton LJ, Offord KP (November 1982). "Postsplenectomy sepsis and mortality in adults". Journal of the American Medical Association. 248 (18): 2279–83. doi:10.1001/jama.248.18.2279. PMID   7131680.
  15. ↑ Cullingford GL, Watkins DN, Watts AD, Mallon DF (June 1991). "Severe late postsplenectomy infection". The British Journal of Surgery. 78 (6): 716–21. doi:10.1002/bjs.1800780626. PMID   2070242. S2CID   23790214.
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