Asplenia causes: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Asplenia}} | {{Asplenia}} | ||
{{CMG}}{{AE}}{{ | {{CMG}} {{AE}} {{Kalpana Giri}} | ||
==Overview== | ==Overview== | ||
Asplenia is caused by either [[congenital]], [[acquired conditions]], or [[functional]]. Common cause include acquired asplenia associated after [[trauma]] or [[surgery]], is one of the commonest cause of the absence of [[splenic tissue]], Functional asplenia include [[diseases]] such as [[sickle cell (SC) disease]], [[hemoglobin SC disease]] and [[sickle beta-thalassemia]], Hyposplenia occurs due to [[medical conditions]] such as [[chronic liver disease]], [[human immunodeficiency syndrome]] ([[HIV]]), [[malignancies]], [[thalassemia]], [[celiac disease]], [[ulcerative colitis]], [[sarcoidosis]], [[amyloidosis]], [[lupus]], [[rheumatoid arthritis]]. Less Common Causes include congenital asplenia may be [[isolated]] or usually seen as a [[clinical syndrome]] such as [[ivemark syndrome]]. | |||
Common | |||
==Causes== | ==Causes== | ||
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===Common Causes=== | ===Common Causes=== | ||
===Acquired=== | ===Acquired=== | ||
[[Image:Situs-ambiguous-asplenia-syndrome-with-bilateral-right-sidedness.jpg|thumb|right|300px|Asplenia syndrome- Case courtesy of A Prof. Essam G Ghonaim, Radiopaedia.org, rID: 37551]] | |||
*'''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> | *'''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 (SC) disease]], [[hemoglobin SC disease]] and [[sickle beta-thalassemia]].<ref name="pmid18564289">{{cite journal| author=Thiruppathy K, Privitera A, Jain K, Gupta S| title=Congenital asplenia and group B streptococcus sepsis in the adult: case report and review of the literature. | journal=FEMS Immunol Med Microbiol | year= 2008 | volume= 53 | issue= 3 | pages= 437-9 | pmid=18564289 | doi=10.1111/j.1574-695X.2008.00422.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18564289 }} </ref> | *'''Functional asplenia''' include [[diseases]] such as [[sickle cell (SC) disease]], [[hemoglobin SC disease]] and [[sickle beta-thalassemia]].<ref name="pmid18564289">{{cite journal| author=Thiruppathy K, Privitera A, Jain K, Gupta S| title=Congenital asplenia and group B streptococcus sepsis in the adult: case report and review of the literature. | journal=FEMS Immunol Med Microbiol | year= 2008 | volume= 53 | issue= 3 | pages= 437-9 | pmid=18564289 | doi=10.1111/j.1574-695X.2008.00422.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18564289 }} </ref> | ||
*'''Hyposplenia''' occurs due to [[medical conditions]] such as [[chronic liver disease]], [[human immunodeficiency syndrome (HIV)]], [[malignancies]], [[thalassemia]], [[celiac disease]], [[ulcerative colitis]], [[sarcoidosis]], [[amyloidosis]], [[lupus]], [[rheumatoid arthritis]].<ref name="pmid32247651">{{cite journal| author=Long B, Koyfman A, Gottlieb M| title=Complications in the adult asplenic patient: A review for the emergency clinician. | journal=Am J Emerg Med | year= 2021 | volume= 44 | issue= | pages= 452-457 | pmid=32247651 | doi=10.1016/j.ajem.2020.03.049 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32247651 }} </ref> | *'''Hyposplenia''' occurs due to [[medical conditions]] such as [[chronic liver disease]], [[human immunodeficiency syndrome (HIV)]], [[malignancies]], [[thalassemia]], [[celiac disease]], [[ulcerative colitis]], [[sarcoidosis]], [[amyloidosis]], [[lupus]], [[rheumatoid arthritis]].<ref name="pmid32247651">{{cite journal| author=Long B, Koyfman A, Gottlieb M| title=Complications in the adult asplenic patient: A review for the emergency clinician. | journal=Am J Emerg Med | year= 2021 | volume= 44 | issue= | pages= 452-457 | pmid=32247651 | doi=10.1016/j.ajem.2020.03.049 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32247651 }} </ref> | ||
===Less Common Causes=== | ===Less Common Causes=== | ||
===Congenital=== | ===Congenital=== | ||
*'''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]].<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> | *'''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]].<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> | ||
*'''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. | *'''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. | ||
*In '''heterotaxy syndrome''' Two human [[genes]], [[connexin 43]] and [[ZIC3]], have been shown to be involved. | *In '''heterotaxy syndrome''' Two human [[genes]], [[connexin 43]] and [[ZIC3]], have been shown to be involved. | ||
*congenital asplenia a very rare anomaly that has been reported in both infants and adults. | *congenital asplenia a very rare anomaly that has been reported in both infants and adults. | ||
Latest revision as of 04:39, 10 September 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Kalpana Giri, MBBS[2]
Overview
Asplenia is caused by either congenital, acquired conditions, or functional. Common cause include acquired asplenia associated after trauma or surgery, is one of the commonest cause of the absence of splenic tissue, Functional asplenia include diseases such as sickle cell (SC) disease, hemoglobin SC disease and sickle beta-thalassemia, Hyposplenia occurs due to medical conditions such as chronic liver disease, human immunodeficiency syndrome (HIV), malignancies, thalassemia, celiac disease, ulcerative colitis, sarcoidosis, amyloidosis, lupus, rheumatoid arthritis. Less Common Causes include congenital asplenia may be isolated or usually seen as a clinical syndrome such as ivemark syndrome.
Causes
Asplenia is caused by either congenital, acquired conditions, or functional.
Common Causes
Acquired
- Acquired asplenia associated after trauma or surgery, is one of the commonest cause of the absence of splenic tissue.[1]
- Functional asplenia include diseases such as sickle cell (SC) disease, hemoglobin SC disease and sickle beta-thalassemia.[2]
- Hyposplenia occurs due to medical conditions such as chronic liver disease, human immunodeficiency syndrome (HIV), malignancies, thalassemia, celiac disease, ulcerative colitis, sarcoidosis, amyloidosis, lupus, rheumatoid arthritis.[3]
Less Common Causes
Congenital
- 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.[4]
- 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.
- In heterotaxy syndrome Two human genes, connexin 43 and ZIC3, have been shown to be involved.
- congenital asplenia a very rare anomaly that has been reported in both infants and adults.
- Infantile cases are almost invariably associated with serious congenital malformations of the cardiovascular, gastrointestinal, and pulmonary systems that are not compatible with long life.
- 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.
- In the adult splenic agenesis is usually an isolated and unexpected finding.
References
- ↑ 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.
- ↑ 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.
- ↑ 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). - ↑ 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.