Asplenia

Asplenia
Classification and external resources
Specialty	medical genetics
ICD-10	D73.0, Q89.0
ICD-9-CM	289.59, 759.01
OMIM	208530 %271400 208540
eMedicine	ped/150 radio/58
Patient UK	Asplenia

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.

Causes

Congenital

• Congenital asplenia is rare. There are two distinct types of genetic disorders: heterotaxy syndrome^[1] and isolated congenital asplenia.^[2]

Acquired

• Acquired asplenia occurs for several reasons:
  • Following splenectomy due to splenic rupture from trauma or because of tumor
  • After splenectomy with the goal of interfering with splenic function, as a treatment for diseases (e.g. idiopathic thrombocytopenic purpura, thalassemia, spherocytosis), in which the spleen's usual activity exacerbates the disease
  • Due to underlying diseases that destroy the spleen (autosplenectomy), e.g. sickle-cell disease.
• Functional asplenia occurs when splenic tissue is present but does not work well, e.g. sickle-cell disease, polysplenia; such patients are managed as if asplenic.

Partial splenectomy and preservation of splenic function

In an effort to preserve some of the spleen's protective roles,^[3] attempts are now often made to preserve a small part of the spleen when performing either surgical subtotal (partial) splenectomy,^[4] or partial splenic embolization.^[5] This may be particularly important in poorer countries where protective measures for patients with asplenia are not available.^[6] However, it has been advised that preoperative vaccination is advisable until the remnant splenic tissue can reestablish its function.^[7]

Risks

Asplenia is a form of immunodeficiency, increasing the risk of sepsis from polysaccharide encapsulated bacteria,^[8] and can result in overwhelming post splenectomy infection (OPSI), often fatal within a few hours. In particular, patients are at risk from Streptococcus pneumoniae, Haemophilus influenzae, and meningococcus.^[8] The risk is elevated as much as 350–fold.^[9]

The risk to asplenic patients has been expressed as equivalent to an adult dying in a road traffic accident (in every 100 people without spleens, 1 to 5 would develop a severe infection per decade) (reference UK Splenectomy Trust Advice)—hence sensible precautions are advisable.^[10] Increased platelet counts can be seen in individuals without a functioning spleen.

Management

To minimise the risks associated with splenectomy, antibiotic and vaccination protocols have been established,^[11][12][13] but are often poorly adhered to by physicians and patients due to the complications resulting from antibiotic prophylaxis such as development of an overpopulation of Clostridium difficile in the intestinal tract.^[14]

Antibiotic prophylaxis

Because of the increased risk of infection, physicians administer oral antibiotics as a prophylaxis after a surgical splenectomy (or starting at birth, for congenital asplenia or functional asplenia).

Those with asplenia are also cautioned to start a full-dose course of antibiotics at the first onset of an upper or lower respiratory tract infection (for example, sore throat or cough), or at the onset of any fever.

Vaccinations

It is suggested that splenectomized persons receive the following vaccinations, and ideally prior to planned splenectomy surgery:

• Pneumococcal polysaccharide vaccine (not before 2 years of age). Children may first need one or more boosters of pneumococcal conjugate vaccine if they did not complete the full childhood series.
• Haemophilus influenzae type b vaccine, especially if not received in childhood. For adults who have not been previously vaccinated, two doses given two months apart was advised in the new 2006 UK vaccination guidelines (in the UK may be given as a combined Hib/MenC vaccine).^[15]
• Meningococcal conjugate vaccine, especially if not received in adolescence. Previously vaccinated adults require a single booster and non-immunised adults advised, in UK since 2006, to have two doses given two months apart.^[15] Children too young for the conjugate vaccine should receive meningococcal polysaccharide vaccine in the interim.^[9]
• Influenza vaccine, every winter, to help prevent getting secondary bacterial infection.

Travel measures

In addition to the normal immunizations advised for the countries to be visited, Group A meningococcus should be included if visiting countries of particular risk (e.g. sub-saharan Africa).^[16] The non-conjugated Meningitis A and C vaccines usually used for this purpose give only 3 years coverage and provide less-effective long-term cover for Meningitis C than the conjugated form already mentioned.^[17]

Those lacking a functional spleen are at higher risk of contracting malaria,^[18] and succumbing to its effects. Travel to malarial areas will carry greater risks and is best avoided. Travellers should take the most appropriate anti-malarial prophylaxis medication and be extra vigilant over measures to prevent mosquito bites.^[11]

The pneumococcal vaccinations may not cover some of the other strains of pneumococcal bacteria present in other countries. Likewise their antibiotic resistance may also vary, requiring a different choice of stand-by antibiotic.

Additional measures

• Surgical and dental procedures - Antibiotic prophylaxis may be required before certain surgical or dental procedures.
• Animal bites - adequate antibiotic cover is required after even minor dog or other animal bites. Asplenic patients are particularly susceptible to infection by capnocytophaga canimorsus and should receive a five-day course of amoxicillin/clavulanate (erythromycin in patients allergic to penicillin).^[19]
• Tick bites - Babesiosis is a rare tickborne infection. Patients should check themselves or have themselves inspected for tick bites if they are in an at-risk situation. Presentation with fever, fatigue, and haemolytic anaemia requires diagnostic confirmation by identifying the parasites within red blood cells on blood film and by specific serology. Quinine (with or without clindamycin) is usually an effective treatment.^[19]
• Alert warning - People without a working spleen can carry a card, or wear a special bracelet or necklet which says that they do not have a working spleen. This would alert a healthcare professional to take rapid action if they become are seriously ill and cannot notify them of their condition.

References

1. Online Mendelian Inheritance in Man. OMIM entry 208530: Right atrial isomerism; RAI. Johns Hopkins University.
2. Online Mendelian Inheritance in Man. OMIM entry 271400: Asplenia, isolated congenital; ICAS. Johns Hopkins University.
3. Grosfeld JL, Ranochak JE (1976). "Are hemisplenectomy and/or primary splenic repair feasible?". J. Pediatr. Surg. 11 (3): 419–24. doi:10.1016/S0022-3468(76)80198-4. PMID 957066.
4. Bader-Meunier B, Gauthier F, Archambaud F, et al. (2001). "Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis". Blood. 97 (2): 399–403. doi:10.1182/blood.V97.2.399. PMID 11154215.
5. Pratl B, Benesch M, Lackner H, et al. (2007). "Partial splenic embolization in children with hereditary spherocytosis". Eur J Haematol. 80 (1): 76–80. doi:10.1111/j.1600-0609.2007.00979.x. PMID 18028435.
6. Sheikha AK, Salih ZT, Kasnazan KH, et al. (2007). "Prevention of overwhelming postsplenectomy infection in thalassemia patients by partial rather than total splenectomy". Can J Surg. 50 (5): 382–6. PMC 2386178. PMID 18031639.
7. Kimber C, Spitz L, Drake D, et al. (1998). "Elective partial splenectomy in childhood". J. Pediatr. Surg. 33 (6): 826–9. doi:10.1016/S0022-3468(98)90651-0. PMID 9660206.
8. Brigden, M. L. (2001). "Detection, education and management of the asplenic or hyposplenic patient". American family physician. 63 (3): 499–506, 508. PMID 11272299.
9. AAP Red Book 2006.
10. "Splenectomy and Infection" (PDF). Splenectomy Trust. March 2002. Archived (PDF) from the original on 2007-09-28. Retrieved 2006-12-12. - reprint from Kent and Medway NHS and Social Care Partnership Trust
11. 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.
12. 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. - published as a response by original authors
13. Davies JM, Barnes R, Milligan D, British Committee for Standards in Haematology - Working Party of the Haematology-Oncology Task Force (2002). "Update of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen" (PDF). Clin Med. 2 (5): 440–3. doi:10.7861/clinmedicine.2-5-440. PMID 12448592.
14. Waghorn DJ (2001). "Overwhelming infection in asplenic patients: current best practice preventive measures are not being followed". J Clin Pathol. 54 (3): 214–8. doi:10.1136/jcp.54.3.214. PMC 1731383. PMID 11253134.
15. Joint Committee on Vaccination and Immunisation (21 December 2006). "Chapter 7 : Immunisation of individuals with underlying medical conditions". In Editors Salisbury D, Ramsay M, Noakes K. Immunisation Against Infectious Disease 2006 (PDF). Edinburgh: Stationery Office. ISBN 0-11-322528-8. - see pages 50-1 and table 7.1
16. "Meningococcal - Children and adults with asplenia or splenic dysfunction" (PDF). Immunization against infectious disease - 'The Green Book' (PDF). 24 August 2009 [2006]. p. 244.
17. Chief Medical Officer (2001). "Meningococcal immunisation for asplenic patients" (PDF). Professional Letter: Chief Medical Officer - Current vaccine and immunization issues. Department of Health. 1: 4. Retrieved 2009-11-07.
18. Boone KE, Watters DA (November 1995). "The incidence of malaria after splenectomy in Papua New Guinea". BMJ. 311 (7015): 1273. doi:10.1136/bmj.311.7015.1273. PMC 2551185. PMID 7496237.
19. "Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen" (PDF). Wilton, Cork, Ireland: Health Service Executive, Southern Area. September 2002.