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| __NOTOC__ | | __NOTOC__ |
| {{Infobox_Disease
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| | Name = Asplenia
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| | Image =
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| | Caption =
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| | DiseasesDB =
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| | ICD10 = {{ICD10|D|73|0|d|70}}, {{ICD10|Q|89|0|q|80}}
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| | ICD9 = {{ICD9|289.59}}, {{ICD9|759.01}}
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| | ICDO =
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| | OMIM = 208530
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| | OMIM_mult = {{OMIM2|%271400}} {{OMIM2|208540}}
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| | MedlinePlus =
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| | MeshID =
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| }}
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| {{Asplenia}} | | {{Asplenia}} |
| {{CMG}}{{AE}} | | {{CMG}}{{AE}} {{Kalpana Giri}} |
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| {{SK}} | | {{SK}} |
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| ==[[Asplenia historical perspective|Historical Perspective]]== | | ==[[Asplenia historical perspective|Historical Perspective]]== |
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| *In 1919, Morris and Bullock provided initial [[experimental]] evidence of the protective role of the [[spleen]] against [[infections]].
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| *In 1952, King and Schumacker reported a series of cases of overwhelming [[post-splenectomy]] [[infections]] (OPSI) caused by [[encapsulated bacteria]].
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| *In 1955, Rowley has demonstrated that [[splenectomized]] human beings fail to respond with a [[significant]] [[rise]] in [[antibody]] [[titer]] when an [[antigen]] is given intravenously.
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| *In 1969, Pearson et al,from USA, was the first to [[discover]] the term [[functional hypoplasia]], a few decades ago when he identified some children suffering from [[sickle cell disease]], who presented with the same [[clinical]] course as in [[splenectomised]] patients.
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| ==[[Asplenia classification|Classification]]== | | ==[[Asplenia classification|Classification]]== |
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| ==[[Asplenia pathophysiology|Pathophysiology]]== | | ==[[Asplenia pathophysiology|Pathophysiology]]== |
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| ===Physiology===
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| 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]].<ref name="pmid21474172">Di Sabatino A, Carsetti R, Corazza GR (2011) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=21474172 Post-splenectomy and hyposplenic states.] ''Lancet'' 378 (9785):86-97. [http://dx.doi.org/10.1016/S0140-6736(10)61493-6 DOI:10.1016/S0140-6736(10)61493-6] PMID: [https://pubmed.gov/21474172 21474172]</ref> 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]].<ref name="pmid25125944">{{cite journal| author=Kirkineska L, Perifanis V, Vasiliadis T| title=Functional hyposplenism. | journal=Hippokratia | year= 2014 | volume= 18 | issue= 1 | pages= 7-11 | pmid=25125944 | doi= | pmc=4103047 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi? dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25125944 }} </ref> 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.
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| ===Pathology===
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| 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]].<ref name="pmid21474172">{{cite journal| author=Di Sabatino A, Carsetti R, Corazza GR| title=Post-splenectomy and hyposplenic states. | journal=Lancet | year= 2011 | volume= 378 | issue= 9785 | pages= 86-97 | pmid=21474172 | doi=10.1016/S0140-6736(10)61493-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21474172 }} </ref> 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]].<ref name="pmid33275684">{{cite journal| author=Lee GM| title=Preventing infections in children and adults with asplenia. | journal=Hematology Am Soc Hematol Educ Program | year= 2020 | volume= 2020 | issue= 1 | pages= 328-335 | pmid=33275684 | doi=10.1182/hematology.2020000117 | pmc=7727556 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33275684 }} </ref>
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| ==[[Asplenia causes|Causes]]== | | ==[[Asplenia causes|Causes]]== |
| Asplenia is caused by either congenital, acquired conditions, or functional.
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| ===Common Causes===
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| ===Acquired===
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| *'''Acquired asplenia''' associated after [[trauma]] or [[surgery]], is one of the commonest cause of the absence of [[splenic tissue]].<ref name="pmid26557043">{{cite journal| author=Erdem SB, Genel F, Erdur B, Ozbek E, Gulez N, Mese T| title=Asplenia in children with congenital heart disease as a cause of poor outcome. | journal=Cent Eur J Immunol | year= 2015 | volume= 40 | issue= 2 | pages= 266-9 | pmid=26557043 | doi=10.5114/ceji.2015.52841 | pmc=4637402 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26557043 }} </ref>
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| *'''Functional asplenia''' include diseases such as [[sickle cell disease]], [[celiac disease]], [[alcoholic liver disease]], [[hepatic cirrhosis]], [[lymphomas]], and [[autoimmune]] disorders.<ref name="pmid25125944">Kirkineska L, Perifanis V, Vasiliadis T (2014) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=25125944 Functional hyposplenism.] ''Hippokratia'' 18 (1):7-11. PMID: [https://pubmed.gov/25125944 25125944]</ref>
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| ===Less Common Causes===
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| ===Congenital===
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| *'''Congenital asplenia''' may be [[isolated]] or usually seen as a [[clinical syndrome]] such as [[ivemark syndrome]]. This [[syndrome]] is classified under [[heterotaxy syndrome]]. It is associated with [[malformation]] of the [[heart]], and abnormal arrangements of organs of the chest and abdomen along with [[asplenia]] or [[hypoplasia]] of the [[spleen]].
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| *'''Isolated asplenia''' are rare and etiology was [[genetic]], due to [[mutations]] in the [[gene RPSA]], which encodes [[ribosomal protein SA]], cause more than half of the cases of [[isolated congenital asplenia]], which was first discovered in 2013.
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| *In '''heterotaxy syndrome''' Two human [[genes]], [[connexin 43]] and [[ZIC3]], have been shown to be involved.
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| *congenital asplenia a very rare anomaly that has been reported in both infants and adults.
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| *'''Infantile''' cases are almost invariably associated with serious congenital malformations of the [[cardiovascular]], [[gastrointestinal]], and [[pulmonary]] systems that are not compatible with long life.
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| *These include [[atrioventricular]] communist, [[pulmonary stenosis]] or [[atresia]], anomalies of the [[aorta]] and [[great vessels]], complete or partial [[situs in versus]], [[anomalies]] of the [[mesenteric]] and [[accessory lobes of the lungs]].
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| *In the '''adult''' [[splenic]] [[agenesis]] is usually an isolated and unexpected finding.<ref name="pmid13322226">{{cite journal| author=MYERSON RM, KOELLE WA| title=Congenital absence of the spleen in an adult; report of a case associated with recurrent Waterhouse-Friderichsen syndrome. | journal=N Engl J Med | year= 1956 | volume= 254 | issue= 24 | pages= 1131-2 | pmid=13322226 | doi=10.1056/NEJM195606142542406 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13322226 }} </ref>
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| ==[[Asplenia differential diagnosis|Differentiating Asplenia from other Diseases]]== | | ==[[Asplenia differential diagnosis|Differentiating Asplenia from other Diseases]]== |
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| ==[[Asplenia epidemiology and demographics|Epidemiology and Demographics]]== | | ==[[Asplenia epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==Risk Factors== | | ==[[Asplenia risk factors|Risk Factors]]== |
| ===Common Risk Factors===
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| *Common risk factors in the development of asplenia include:
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| **[[Trauma]] <ref name="pmid26557043">{{cite journal| author=Erdem SB, Genel F, Erdur B, Ozbek E, Gulez N, Mese T| title=Asplenia in children with congenital heart disease as a cause of poor outcome. | journal=Cent Eur J Immunol | year= 2015 | volume= 40 | issue= 2 | pages= 266-9 | pmid=26557043 | doi=10.5114/ceji.2015.52841 | pmc=4637402 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26557043 }} </ref>
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| **[[Atraumatic]] indication for [[splenectomy]] includes:<ref name="pmid27018168">{{cite journal| author=Browning MG, Bullen N, Nokes T, Tucker K, Coleman M| title=The evolving indications for splenectomy. | journal=Br J Haematol | year= 2017 | volume= 177 | issue= 2 | pages= 321-324 | pmid=27018168 | doi=10.1111/bjh.14060 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27018168 }} </ref>
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| ***[[malignancy]]
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| ***[[hematological autoimmune disorder]]
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| ****[[Idiopathic Thrombocytopenic Purpura (ITP)]]
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| ****[[Autoimmune Hemolytic Anemia (AIHA)]]
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| **[[Surgery]]: includes
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| ***[[unexplained splenomegaly]]
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| ***[[autoimmune]]
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| ***[[malignant]]
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| ===Less Common Risk Factors===
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| *Less common risk factor include:
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| **[[mutations]] in the [[gene RPSA]], is a risk factor for [[Isolated asplenia]].<ref name="pmid25840456">{{cite journal| author=Bolze A| title=[Connecting isolated congenital asplenia to the ribosome]. | journal=Biol Aujourdhui | year= 2014 | volume= 208 | issue= 4 | pages= 289-98 | pmid=25840456 | doi=10.1051/jbio/2015001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25840456 }} </ref>
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| **Two human [[genes]], [[connexin 43]] and [[ZIC3]], is a risk factor for [[heterotaxy syndrome]].<ref name="pmid19618213">{{cite journal| author=Ahmed SA, Zengeya S, Kini U, Pollard AJ| title=Familial isolated congenital asplenia: case report and literature review. | journal=Eur J Pediatr | year= 2010 | volume= 169 | issue= 3 | pages= 315-8 | pmid=19618213 | doi=10.1007/s00431-009-1030-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19618213 }} </ref>
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| ==[[Asplenia screening|Screening]]== | | ==[[Asplenia screening|Screening]]== |
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| ==[[Asplenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | | ==[[Asplenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| ===Natural History===
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| *If left untreated, Patients with asplenia or hyposplenia are at risk of life-threatening infection.
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| *Overwhelming post-splenectomy infection (OPSI) occurs in 5% of patients and has a mortality rate of 38%–70%.
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| *Patients with functional asplenia and hyposplenia who have not undergone a splenectomy can present with a life-threatening infection comparable to an OPSI
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| ==Diagnosis== | | ==Diagnosis== |
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| ==Treatment== | | ==Treatment== |
| ==Primary prevention==
| | [[Asplenia medical treatment|Medical Therapy]] | [[TAsplenia surgical techniques|Surgery]] | [[Asplenia primary prevention|Primary Prevention]] | [[Asplenia secondary prevention|Secondary Prevention]] | [[Asplenia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Asplenia future or investigational therapies|Future or Investigational Therapies]] |
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| *Vaccination against these encapsulated bacteria is recommended to prevent asplenia patients from severe infection. Up to 87% of asplenic patients were found to have been infected with Streptococcus pneumoniae, one of the most common bacterial pathogen leading to infection in patients with asplenia.
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| *[[Vaccinations]] are also recommended before [[splenectomy]] and after the surgical removal. For those with [[functional asplenia]] or [[autosplenectomy]], it is also advised to continue aggressive [[vaccination schedules]]. It is recommended that patients should be given the [[pneumococcal conjugate vaccine (PCV-13)]] 8 weeks in advance, as well as the [[pneumococcal polysaccharide vaccine (PPSV-23)]], [[Haemophilus influenzae type B vaccine (Hib)]], and the [[quadrivalent meningococcal]] [[conjugate vaccine]] 14 days before planned surgery for [[splenectomy]].
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| ==Secondary prevention==
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| Effective measures for the secondary prevention of asplenia include:
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| *[[Patient]] should carry an [[alert card]] or [[bracelet]] and an [[up-to-date]] [[vaccination record]].<ref name="pmid32759171">{{cite journal| author=O'Neill NE, Baker J, Ward R, Johnson C, Taggart L, Sholzberg M| title=The development of a quality improvement project to improve infection prevention and management in patients with asplenia or hyposplenia. | journal=BMJ Open Qual | year= 2020 | volume= 9 | issue= 3 | pages= | pmid=32759171 | doi=10.1136/bmjoq-2019-000770 | pmc=7410002 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32759171 }} </ref>
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| *Adult with [[asplenia]], if unable to seek [[medical attention]] within 2 hours, should have [[access]] to [[preprescribed antibiotics]] which should be taken at [[fever]] onset.
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| *The risk of [[infection]] can be significantly reduced by using [[systematic]], [[long-term approaches]] to care for [[asplenic patients]].
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| *[[Patient]] and [[family education program]] that addresses the [[risk]] of [[infection]] in these at-risk [[patients]].<ref name="pmid33275684">{{cite journal| author=Lee GM| title=Preventing infections in children and adults with asplenia. | journal=Hematology Am Soc Hematol Educ Program | year= 2020 | volume= 2020 | issue= 1 | pages= 328-335 | pmid=33275684 | doi=10.1182/hematology.2020000117 | pmc=7727556 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=33275684 }} </ref>
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| ==Case Studies== | | ==Case Studies== |
| [[Asplenia case study one|Case #1]] | | [[Asplenia case study one|Case #1]] |
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| {{Hematology}}
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| {{Phakomatoses and other congenital malformations not elsewhere classified}}
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| [[de:Asplenie]]
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| [[nl:Asplenie]]
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| [[fi:Asplenia]]
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}}
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| [[Category:Medicine]] | | [[Category:Medicine]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
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| [[Category:Immunology]] | | [[Category:Immunology]] |
| [[Category:Hematology]] | | [[Category:Hematology]] |
| <references />
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