Spleen: Disorders

Ernest Kuchar, Medical University of Warsaw, Warsaw, Poland
Monika Karlikowska‐Skwarnik, Wrocław Medical University, Wrocław, Poland

Published online: May 2017

DOI: 10.1002/9780470015902.a0002183.pub2

Abstract

The spleen is an important peripheral immunologic organ although not essential for life. The main functions of the spleen are filtering of blood, lymphopoiesis and immunoglobulin production. The spleen is abundantly supplied with blood, which the other secondary lymphoid organs do not have, and clears particulate material, for example, opsonised bacteria from the blood. Reduced function, known as functional asplenia, is observed in many various pathological conditions, for example, when the spleen is congested with blood or when infiltrated by metastatic tumours or contains large haemangiomas. Most common causes of splenic hypofunction are coeliac disease, sprue, sickle cell disease and haematologic disorders. Individuals with removed spleen or impaired splenic function are prone to serious and often fatal bacterial infections. The vast majority of these serious infections, caused by encapsulated bacteria (pneumococci, meningococci and Haemophilus influenzae type b) can be effectively prevented by immunisations and antibiotic prophylaxis.

Key Concepts

  • Spleen is an important secondary immunologic organ located in the left epigastrium beneath the lower rib cage, responsible for blood filtration and antibodies production.
  • Spleen is important but not essential for life and can be removed.
  • Reduced spleen function, known as functional asplenia, is observed in various pathological conditions including sickle cell disease, celiac disease and haematologic disorders.
  • Spleen dysfunction is associated with increased risk of serious and sometimes fatal bacterial sepsis or meningitis, usually caused by pneumococci and other capsular bacteria.
  • The vast majority of bacterial infections in asplenic individuals can be prevented by currently available immunisations and prophylactic antibiotics.

Keywords: splenectomy; impaired immunity; overwhelming post‐splenectomy infection (OPSI); immune system; immunodeficiency; lymphatic system; vaccination

Figure 1. Basic anatomy of the spleen. Reproduced from U. S. National Institutes of Health, National Cancer Institute.
Figure 2. Transverse section of a portion of the spleen. Reproduced from Gray's Anatomy (1918) – copyrights expired.
Figure 3. The spleen contains two different tissues, white pulp (A) and red pulp (B). The white pulp functions in producing and growing immune and blood cells. The red pulp functions in filtering blood of antigens, microorganisms and defective or worn‐out red blood cells. Reproduced from Wikipedia, file licensed under the Creative Commons Attribution‐Share Alike 4.0 International license.
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References

American Academy of Pediatrics (2015) Immunization in immunocompromised children. In: Kimberlin DW , Brady MT , Jackson MA and Long SS (eds) Red Book: 2015 Report of the Committee on Infectious Diseases, 30th edn, p. 74. Elk Grove Village, IL: American Academy of Pediatrics.

Backhus LM and Bremner RM (2006) Images in clinical medicine. Intrathoracic splenosis after remote trauma. New England Journal of Medicine 355: 1811.

Balazs M , Martin F , Zhou T and Kearney J (2002) Blood dendritic cells interact with splenic marginal zone B cells to initiate T‐independent immuneresponses. Immunity 17 (3): 341–352.

Bolze A , Mahlaoui N , Byun M , et al. (2013) Ribosomal protein SA haploinsufficiency in humans with isolated congenital asplenia. Science 340: 976–978.

Brousse V , Buffet P and Rees D (2014) The spleen and sickle cell disease: the sick(led) spleen. British Journal of Haematology 166 (2): 165–176.

Buffet P , Milon G , Brousse V , et al. (2006) Ex vivo perfusion of human spleens maintains clearing and processing functions. Blood 107 (9): 3745–3752.

Cerutti A , Cols M and Puga I (2013) Marginal zone B cells: virtues of innate like antibody‐producing lymphocytes. Nature Reviews Immunology 13: 118–132.

Corazza GR , Zoli G , Di Sabatino A , Ciccocioppo R and Gasbarrini G (1999) A reassessment of splenic hypofunction in celiac disease. The American Journal of Gastroenterology 94: 391–397.

Craddock CG , Longmire R and McMillan R (1971) Lymphocytes and the immune function. New England Journal of Medicine 285: 378–384.

Crosby WH (1959) Normal functions of the spleen relative to the red blood cells. Blood 14: 399–408.

Di Sabatino A , Carsetti R and Corazza G (2011a) Post‐splenectomy and hyposplenic states. The Lancet 378 (9785): 86–97.

Eraklis AJ , Kevy S , Diamond LK and Gross RE (1967) Overwhelming infections after splenectomy. New England Journal of Medicine 276: 1225–1229.

Fishman D and Isenberg D (1997) Splenic involvement in rheumatic diseases. Seminars in Arthritis and Rheumatism 27 (3): 141–155.

Gielchinsky Y , Elstein D , Hadas‐Halpern I , et al. (1999) Is there a correlation between degree of splenomegaly, symptoms and hypersplenism? A study of 218 patients with Gaucher disease. British Journal of Haematology 106 (3): 812–816.

Holdsworth RJ , Irving AD and Cuschieri A (1991) Postsplenectomy sepsis and its mortality rate: actual versus perceived risks. British Journal of Surgery 78: 1031–1038.

Horan M and Colebatch JH (1962) Relationship between splenectomy and subsequent infections. Archives of Disease in Childhood 97: 398–402.

Ivemark BI (1955) Implications of agenesis of the spleen on the pathogenesis of the conotruncus anomalies in childhood. Acta Paediatrica 44 (supplement 104): 1–110.

Kaza RK , Azar S , Al‐Hawary M and Francis I (2010) Primary and secondary neoplasms of the spleen. Cancer Imaging 10 (1): 173–182.

Kim CH (2010) Homeostatic and pathogenic extramedullary hematopoiesis. Journal of Blood Medicine 1: 13–19.

Kuchar E , Miśkiewicz K and Karlikowska M (2015a) A review of guidance on immunization in persons with defective or deficient splenic function. British Journal of Haematology 171: 683–694.

Lecturio (2016) The Spleen – Central Organ of the Lymphatic System. https://www.lecturio.com/magazine/spleen/

Markus HS and Toghill PJ (1991) Impaired splenic function in elderly people. Age and Ageing 20 (4): 287–290.

Micelli AB (1994) Splenogonadal fusion; a rare cause of a scrotal mass. British Journal of Urology 74: 250.

Morris D and Bullock F (1919) The importance of the spleen in resistance to infection. Annals of Surgery 70 (5): 513–521.

Muller A , Cornford E and Toghill PJ (1993) Splenic function in inflammatory bowel disease: assessment by differential interference microscopy and splenic ultrasound. The Quarterly Journal of Medicine 86 (5): 333–340.

Nores M , Phillips EH , Morgenstern L and Hiatt JR (1998) The clinical spectrum of splenic infarction. American Surgery 64: 182.

OMIM (2016) #208530 RIGHT ATRIAL ISOMERISM; RAI. http://www.omim.org/entry/208530?search=ivemark%20syndrome&highlight=syndromic%20ivemark%20syndrome#18

Pearson HA , Spencer RP and Cornelius EA (1969) Functional asplenia in sickle cell anemia. New England Journal of Medicine 281: 923–926.

Pearson HA , Johnston D , Smith KA and Touloukian RJ (1978) The born‐again spleen; return of splenic function after splenectomy for trauma. New England Journal of Medicine 298: 1389–1392.

Piliero P and Furie R (1990) Functional asplenia in systemic lupus erythematosus. Seminars in Arthritis and Rheumatism 20 (3): 185–189.

Rialon KL , Englum BR , Gulack BC , et al. (2016) Comparative effectiveness of treatment strategies for severe splenic trauma in the pediatric population. Am J Surg 212: 786.

Roberts CWM , Shutter JR and Korsmeyer SJ (1994) Hox11 controls the genesis of the spleen. Nature 368: 1451.

Robinson RI , Bullen AW , Hall R , et al. (1980) Splenic size and functions in adult coeliac disease. British Journal of Radiology 52: 532–537.

Rubin L , Levin M , Ljungman P , et al. (2014) 2013 IDSA clinical practice guideline for vaccination of the immunocompromised host. Clinical Infectious Diseases 58 (3): 309–318.

Singer DB (1973) Post splenectomy sepsis. Pediatric Pathology 1: 285–312.

Spencer RP and Pearson HA (1975) Radionuclide Studies of the Spleen. Cleveland: CRC Press.

Thipphavong S , Duigenan S , Schindera ST , et al. (2014) Nonneoplastic, benign, and malignant splenic diseases: cross‐sectional imaging findings and rare disease entities. AJR American Journal of Roentgenology 203: 315.

Ting W , Silverman NA , Arzouman DA and Levitsky S (1990) Splenic septic emboli in endocarditis. Circulation 82 (5 Suppl:IV): 105–109.

Wardemann H , Boehm T , Dear N and Carsetti R (2002) B‐1a B cells that link the innate and adaptive immune responses are lacking in the absence of the spleen. The Journal of Experimental Medicine 195: 771–780.

William B and Corazza G (2007) Hyposplenism: a comprehensive review. Part I: basic concepts and causes. Hematology 12 (1): 1–13.

Yuste JR , Buades J , Guillen EF and Vivas I (2014) Posttraumatic intrathoracic splenosis: from clinical suspicion to noninvasive diagnosis. Am J Med 127: e3.

Zhou ZH , Tzioufas AG and Notkins AL (2007) Properties and function of polyreactive antibodies. Journal of Autoimmunity 29: 219–228.

Further Reading

Di Sabatino A , Carsetti R and Corazza G (2011b) Post‐splenectomy and hyposplenic states. The Lancet 378 (9785): 86–97.

Feder HM and Pearson HA (1999b) Assessment of splenic function in familial asplenia. New England Journal of Medicine 341: 210–211.

Kuchar E , Miśkiewicz K and Karlikowska M (2015b) A review of guidance on immunization in persons with defective or deficient splenic function. British Journal of Haematology 171: 683–694.

Pearson HA (1998) The spleen and disturbances of splenic function. In: Nathan DG and Orkin SH (eds) Hematology of Infancy and Childhood, 5th, chap 26, edn, pp. 1051–1068. Philadelphia: WB Saunders.

Related Sub-Topics:

Infection | Tissues of the Immune System
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Article Title: Spleen: Disorders
 
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Kuchar, Ernest, and Karlikowska‐Skwarnik, Monika(May 2017) Spleen: Disorders. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0002183.pub2]