Asplenia

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Asplenia
ICD-10 D73.0, Q89.0
ICD-9 289.59, 759.01
OMIM 208530 %271400 208540

Asplenia Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Asplenia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

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Treatment

Medical Therapy

Interventions

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Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief:

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Overview

Historical Perspective

Classification

Pathophysiology

Physiology

The spleen consists of three functional inter-related compartments: red pulp, white pulp, marginal zone. The red pulp is a sponge-like structure filled with blood flowing through sinuses and cords functions as a filter for blood elements.[2] The white pulp consists primarily of lymphatic tissue creating structures called germinal centers which contain lymphocytes (activated B-lymphocytes among others), macrophages, and dendritic cells. They are situated in direct contact with splenic arterioles, branches of the splenic artery. Another region of the white pulp is that the periarteriolar lymphatic sheath, which consists of nodules containing mostly B lymphocytes. The marginal zone surrounds the white pulp and consists of blood vessels, macrophages, and specialized B cells.[4] The primary physiologic role of spleen is the filtration and processing of senescent blood cells, predominantly red blood cells and immunologically helps protect against encapsulated microorganisms and response to infectious pathogens. It contains both hematopoietic and lymphopoietic elements, which provides a basis for extramedullary hematopoiesis when necessary.

Pathology

The spleen plays integral roles in the immune system and reticuloendothelial systems. It also modulates the inflammatory and coagulation cascades.[5] It is understood that Asplenia is a variety of clinical settings, and it can refer to an anatomic absence of the spleen or functional asplenia secondary to a variety of disease states. The absence of a spleen is a well-known risk factor for severe bacterial infections, especially due to encapsulated bacteria. The spleen contains 2 types of tissues: white pulp and red pulp. The white pulp is rich in T-cell lymphocytes, naïve B-cell lymphocytes, and macrophages. The antigen-presenting cells (APC) can enter the white pulp and activate T cells, which in turn activate naïve B cells and differentiate into plasma cells that generate immunoglobulin M antibodies followed by immunoglobulin G antibodies. B cells can also act as antigen-presenting cells and has a phagocytic function to help opsonize encapsulated bacteria. About half of the total B cells in the blood express the memory marker CD27 and carry somatic mutations, and are therefore thought to be memory B cells. There are two types of memory B cells in human beings: switched memory B cells and IgM memory B cells. Switched memory B cells, which are the final product of germinal center reactions, produce high-affinity antibodies and have a protective function against infection. IgM memory B cells, need the spleen for their survival and generation and have the ability to produce natural antibodies. They also produce antibodies against Streptococcus pneumonia, Neisseria meningitidis, and Haemophilus influenzae type b. They can initiate T-cell-independent immune responses on infection or vaccination with capsular polysaccharide antigens.[2] The red pulp has macrophages and is responsible for filtering damaged, older red blood cells as well as phagocytosing opsonized bacteria. Due to this role of removing damaged erythrocytes, the spleen also plays an important role in the defense against intraerythrocytic parasitic infections such as malaria and Babesia.[6] About 30% of platelets are sequestrated in the splenic tissue, spleen is the main site of storage of circulating platelels. [4]

Functional asplenia is associated with sickle cell anemia, hemoglobin sickle cell disease, and sickle cell hemoglobin β thalassemia. Patient with these hemoglobinopathies starts losing a splenic function, where the spleen is initially enlarged due to excessive red cell entrapment results in atrophy and degeneration in advanced disease. This atrophy is called autosplenectomy and may be consequent] to multiple acute episodes of entrapment of massive red cell volumes in the splenic tissue, followed by splenic infarctions. Functional hyposplenism associated with celiac disease and inflammatory bowel disease leads to spleen’s reticuloendothelial atrophy due to loss of lymphocytes through the inflamed enteric mucosa. Hyposplenism in autoimmune disorders one of the major mechanisms could be reticuloendothelial block due to circulating [[immune complexes]. In hematologic and neoplastic disorders, it is probably due to splenic tissue infiltration by tumor cells or due to vascular occlusion. Hyposplenism in hepatic disorders, might be caused by disruption of normal hepatic microcirculation due to portal hypertension. In acute or chronic alcohol consumption, direct toxic effect of alcohol is implied in all disorders.[4]

Causes

Asplenia is caused by either congenital, acquired conditions, or functional.

Common Causes

Acquired

Less Common Causes

Congenital

Differentiating Asplenia from other Diseases

Epidemiology and Demographics

Incidence

Prevalence

Age

Risk Factors

Common Risk Factors

Less Common Risk Factors

Screening

screening for asplenia is by the detection of Howell-Jolly bodies (ie, erythrocytes with nuclear remnants) is recommended. [16]

Natural History, Complications and Prognosis

Natural History

Complications

Common complications of asplenia include:

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Interventions | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Medical Therapy

Emergency Medical Management of suspected sepsis in Asplenic patient

Asplenia can cause sepsis and require immediate management:[18]

Surgery

The mainstay of treatment for asplenia is medical therapy and prevention.[20]

Primary prevention

Vaccination

Antibiotic Prophylaxis

Malaria Prophylaxis

Secondary prevention

Effective measures for the secondary prevention of asplenia include:

Case Studies

Case #1

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Template:WikiDoc Sources

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  2. 2.0 2.1 2.2 2.3 2.4 Di Sabatino A, Carsetti R, Corazza GR (2011). "Post-splenectomy and hyposplenic states". Lancet. 378 (9785): 86–97. doi:10.1016/S0140-6736(10)61493-6. PMID 21474172.
  3. Fachet J, Foris G (1975). "Enodotoxin-induced non-specific resistance to Trypanosoma equiperdum in neonatally thymectomized or splenectomized Wistar rats". Keio J Med. 24 (4): 347–53. doi:10.2302/kjm.24.347. PMID 1228266.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 Kirkineska L, Perifanis V, Vasiliadis T (2014). "Functional hyposplenism". Hippokratia. 18 (1): 7–11. PMC 4103047. PMID 25125944.
  5. 5.0 5.1 5.2 5.3 Long B, Koyfman A, Gottlieb M (2021). "Complications in the adult asplenic patient: A review for the emergency clinician". Am J Emerg Med. 44: 452–457. doi:10.1016/j.ajem.2020.03.049. PMID 32247651 Check |pmid= value (help).
  6. 6.0 6.1 Lee GM (2020). "Preventing infections in children and adults with asplenia". Hematology Am Soc Hematol Educ Program. 2020 (1): 328–335. doi:10.1182/hematology.2020000117. PMC 7727556 Check |pmc= value (help). PMID 33275684 Check |pmid= value (help).
  7. 7.0 7.1 Erdem SB, Genel F, Erdur B, Ozbek E, Gulez N, Mese T (2015). "Asplenia in children with congenital heart disease as a cause of poor outcome". Cent Eur J Immunol. 40 (2): 266–9. doi:10.5114/ceji.2015.52841. PMC 4637402. PMID 26557043.
  8. Thiruppathy K, Privitera A, Jain K, Gupta S (2008). "Congenital asplenia and group B streptococcus sepsis in the adult: case report and review of the literature". FEMS Immunol Med Microbiol. 53 (3): 437–9. doi:10.1111/j.1574-695X.2008.00422.x. PMID 18564289.
  9. MYERSON RM, KOELLE WA (1956). "Congenital absence of the spleen in an adult; report of a case associated with recurrent Waterhouse-Friderichsen syndrome". N Engl J Med. 254 (24): 1131–2. doi:10.1056/NEJM195606142542406. PMID 13322226.
  10. Hansen K, Singer DB (2001). "Asplenic-hyposplenic overwhelming sepsis: postsplenectomy sepsis revisited". Pediatr Dev Pathol. 4 (2): 105–21. doi:10.1007/s100240010145. PMID 11178626.
  11. LIPSON RL, BAYRD ED, WATKINS CH (1959). "The postsplenectomy blood picture". Am J Clin Pathol. 32: 526–32. doi:10.1093/ajcp/32.6.526. PMID 14417436.
  12. 12.0 12.1 Holdsworth RJ, Irving AD, Cuschieri A (1991). "Postsplenectomy sepsis and its mortality rate: actual versus perceived risks". Br J Surg. 78 (9): 1031–8. doi:10.1002/bjs.1800780904. PMID 1933181.
  13. Browning MG, Bullen N, Nokes T, Tucker K, Coleman M (2017). "The evolving indications for splenectomy". Br J Haematol. 177 (2): 321–324. doi:10.1111/bjh.14060. PMID 27018168.
  14. Bolze A (2014). "[Connecting isolated congenital asplenia to the ribosome]". Biol Aujourdhui. 208 (4): 289–98. doi:10.1051/jbio/2015001. PMID 25840456.
  15. Ahmed SA, Zengeya S, Kini U, Pollard AJ (2010). "Familial isolated congenital asplenia: case report and literature review". Eur J Pediatr. 169 (3): 315–8. doi:10.1007/s00431-009-1030-0. PMID 19618213.
  16. Corazza GR, Ginaldi L, Zoli G, Frisoni M, Lalli G, Gasbarrini G; et al. (1990). "Howell-Jolly body counting as a measure of splenic function. A reassessment". Clin Lab Haematol. 12 (3): 269–75. doi:10.1111/j.1365-2257.1990.tb00037.x. PMID 2125541.
  17. Hale AJ, LaSalvia M, Kirby JE, Kimball A, Baden R (2016). "Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult". IDCases. 6: 1–4. doi:10.1016/j.idcr.2016.08.004. PMC 4995527. PMID 27583208.
  18. 18.0 18.1 Salvadori MI, Price VE, Canadian Paediatric Society, Infectious Diseases and Immunization Committee (2014). "Preventing and treating infections in children with asplenia or hyposplenia". Paediatr Child Health. 19 (5): 271–8. PMC 4029242. PMID 24855431.
  19. Rhodes A, Evans LE, Alhazzani W, Levy MM, Antonelli M, Ferrer R; et al. (2017). "Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock: 2016". Intensive Care Med. 43 (3): 304–377. doi:10.1007/s00134-017-4683-6. PMID 28101605.
  20. 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.
  21. Huebner ML, Milota KA (2015). "Asplenia and fever". Proc (Bayl Univ Med Cent). 28 (3): 340–1. doi:10.1080/08998280.2015.11929267. PMC 4462215. PMID 26130882.
  22. Quéffélec C, Billet L, Duffau P, Lazaro E, Machelart I, Greib C; et al. (2020). "Prevention of infection in asplenic adult patients by general practitioners in France between 2013 and 2016 : Care for the asplenic patient in general practice". BMC Fam Pract. 21 (1): 163. doi:10.1186/s12875-020-01237-3. PMC 7425533 Check |pmc= value (help). PMID 32787857 Check |pmid= value (help).
  23. Committee to Advise on Tropical Medicine and Travel (CATMAT) (2009). "Canadian recommendations for the prevention and treatment of malaria among international travellers--2009". Can Commun Dis Rep. 35 Suppl 1: 1–82. PMID 19750611.
  24. O'Neill NE, Baker J, Ward R, Johnson C, Taggart L, Sholzberg M (2020). "The development of a quality improvement project to improve infection prevention and management in patients with asplenia or hyposplenia". BMJ Open Qual. 9 (3). doi:10.1136/bmjoq-2019-000770. PMC 7410002 Check |pmc= value (help). PMID 32759171 Check |pmid= value (help).