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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]]== |
| *In 1919, Morris and Bullock provided initial [[experimental]] evidence of the protective role of the [[spleen]] against [[infections]].<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>
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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.<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 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>
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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. <ref name="pmid30844198">{{cite journal| author=| title=StatPearls | journal= | year= 2021 | volume= | issue= | pages= | pmid=30844198 | doi= | pmc= | url= }} </ref> The absence of a spleen is a well-known risk factor for severe bacterial infections, especially due to encapsulated bacteria.<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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| ==[[Asplenia causes|Causes]]== | | ==[[Asplenia causes|Causes]]== |
| Asplenia is caused by either congenital, functional, or acquired conditions.
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| *'''Congenital asplenia'''
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| **Is 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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| *'''Acquired asplenia''' associated after [[trauma]] or [[surgery]].<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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| ==[[Asplenia differential diagnosis|Differentiating Asplenia from other Diseases]]== | | ==[[Asplenia differential diagnosis|Differentiating Asplenia from other Diseases]]== |
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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== |
| [[Asplenia medical therapy|Medical Therapy]] | [[Asplenia interventions|Interventions]] | [[Asplenia surgery|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]] | | [[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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| ==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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| [[fi:Asplenia]]
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
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| [[Category:Medicine]] | | [[Category:Medicine]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |