|
|
| (43 intermediate revisions by 2 users not shown) |
| Line 1: |
Line 1: |
| __NOTOC__ | | __NOTOC__ |
| {{Infobox_Disease
| |
| | Name = Asplenia
| |
| | Image =
| |
| | Caption =
| |
| | DiseasesDB =
| |
| | ICD10 = {{ICD10|D|73|0|d|70}}, {{ICD10|Q|89|0|q|80}}
| |
| | ICD9 = {{ICD9|289.59}}, {{ICD9|759.01}}
| |
| | ICDO =
| |
| | OMIM = 208530
| |
| | OMIM_mult = {{OMIM2|%271400}} {{OMIM2|208540}}
| |
| | MedlinePlus =
| |
| | MeshID =
| |
| }}
| |
| {{Asplenia}} | | {{Asplenia}} |
| {{CMG}}{{AE}} | | {{CMG}}{{AE}} {{Kalpana Giri}} |
|
| |
|
| {{SK}} | | {{SK}} |
| Line 21: |
Line 8: |
|
| |
|
| ==[[Asplenia historical perspective|Historical Perspective]]== | | ==[[Asplenia historical perspective|Historical Perspective]]== |
|
| |
| *In 421 BC, [[Hippocrates]] made the first description of the [[gross anatomy]] of the [[spleen]].<ref name="pmid17364987">{{cite journal| author=William BM, Corazza GR| title=Hyposplenism: a comprehensive review. Part I: basic concepts and causes. | journal=Hematology | year= 2007 | volume= 12 | issue= 1 | pages= 1-13 | pmid=17364987 | doi=10.1080/10245330600938422 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17364987 }} </ref>
| |
| *In 360 BC, [[Plato]], [[described]] the [[spleen]] as been constructed "with a view of keeping the [[liver]] [[bright]] and [[pure]].
| |
| *In 1899, [[Chauffard]] described that [[increased]] [[splenic]] activity is [[linked]] to [[hemolysis]], and in 1910, [[Sutherland and Brughard]] performed the first [[therapeutic splenectomy]] in a patient with hereditary spherocytosis.
| |
| *In 1913, [[Eppinger]] was the first to [[introduced]] the term [[hyposplenism]] to describe the [[post-splenectomy state]].
| |
| *In 1916, [[Kaznelson]] performed [[therapeutic splenectomy]] in a patient with [[idiopathic]] [[thrombocytopenic purpura]].
| |
| *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>
| |
| *In 1935, [[Diggs]] provide a [[histological]] description of the [[spleen]] in [[sickle cell anemia]].<ref name="pmid17364987">{{cite journal| author=William BM, Corazza GR| title=Hyposplenism: a comprehensive review. Part I: basic concepts and causes. | journal=Hematology | year= 2007 | volume= 12 | issue= 1 | pages= 1-13 | pmid=17364987 | doi=10.1080/10245330600938422 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17364987 }} </ref>
| |
| *In 1952, King and Schumacker reported a series of cases of overwhelming [[post-splenectomy]] [[infections]] (OPSI) caused by [[encapsulated bacteria]].<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>
| |
| *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="pmid1228266">{{cite journal| author=Fachet J, Foris G| title=Enodotoxin-induced non-specific resistance to Trypanosoma equiperdum in neonatally thymectomized or splenectomized Wistar rats. | journal=Keio J Med | year= 1975 | volume= 24 | issue= 4 | pages= 347-53 | pmid=1228266 | doi=10.2302/kjm.24.347 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1228266 }} </ref>
| |
| *In 1955, Dameshek coined the term [[hyposplenism]] to describe a patient with [[coeliac disease]].<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>
| |
| *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.<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>
| |
|
| |
|
| ==[[Asplenia classification|Classification]]== | | ==[[Asplenia classification|Classification]]== |
|
| |
|
| ==[[Asplenia pathophysiology|Pathophysiology]]== | | ==[[Asplenia pathophysiology|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]].<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.
| |
|
| |
| ===Pathology===
| |
| The [[spleen]] plays [[integral roles]] in the [[immune system]] and [[reticuloendothelial systems]]. It also [[modulates]] the [[inflammatory]] and [[coagulation cascades]].<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> 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>
| |
|
| |
|
| ==[[Asplenia causes|Causes]]== | | ==[[Asplenia causes|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]].<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>
| |
|
| |
| *'''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>
| |
| ===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]].
| |
| *'''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.<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>
| |
|
| |
|
| ==[[Asplenia differential diagnosis|Differentiating Asplenia from other Diseases]]== | | ==[[Asplenia differential diagnosis|Differentiating Asplenia from other Diseases]]== |
| Line 69: |
Line 20: |
|
| |
|
| ==[[Asplenia risk factors|Risk Factors]]== | | ==[[Asplenia risk factors|Risk Factors]]== |
| ===Common Risk Factors===
| |
|
| |
| *Common risk factors in the development of asplenia include:
| |
| **[[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>
| |
| **[[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>
| |
| ***[[malignancy]]
| |
| ***[[hematological autoimmune disorder]]
| |
| ****[[Idiopathic Thrombocytopenic Purpura (ITP)]]
| |
| ****[[Autoimmune Hemolytic Anemia (AIHA)]]
| |
| **[[Surgery]]: includes
| |
| ***[[unexplained splenomegaly]]
| |
| ***[[autoimmune]]
| |
| ***[[malignant]]
| |
|
| |
| ===Less Common Risk Factors===
| |
|
| |
| *Less common risk factor include:
| |
| **[[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>
| |
| **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>
| |
|
| |
|
| ==[[Asplenia screening|Screening]]== | | ==[[Asplenia screening|Screening]]== |
| [[screening]] for [[asplenia]] is by the [[detection]] of [[Howell-Jolly bodies]] (ie, [[erythrocytes]] with [[nuclear remnants]]) is recommended.
| |
| <ref name="pmid2125541">{{cite journal| author=Corazza GR, Ginaldi L, Zoli G, Frisoni M, Lalli G, Gasbarrini G | display-authors=etal| title=Howell-Jolly body counting as a measure of splenic function. A reassessment. | journal=Clin Lab Haematol | year= 1990 | volume= 12 | issue= 3 | pages= 269-75 | pmid=2125541 | doi=10.1111/j.1365-2257.1990.tb00037.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2125541 }} </ref>
| |
|
| |
|
| ==[[Asplenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | | ==[[Asplenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| ===Natural History===
| |
|
| |
| *If left [[untreated]], [[Patients]] with [[asplenia]] or [[hyposplenia]] are at risk of [[life-threatening]] [[infection]].<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>
| |
| *[[Overwhelming post-splenectomy infection]] (OPSI) occurs in [[5%]] of [[patients]] and has a [[mortality rate]] of [[38%–70%]].
| |
| *Functional asplenia is most common in [[sickle cell disease]] and [[occurs]] within the [[first 3-5 years]] of [[life]].<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>
| |
| **Patients with [[functional asplenia]] and [[hyposplenia]] who have not [[undergone]] a [[splenectomy]] can present with a [[life-threatening]] [[infection]] [[comparable]] to an [[OPSI]].
| |
|
| |
| ===Complications===
| |
| Common complications of [disease name] include:
| |
| *[[overwhelming post-splenectomy infection (OPSI)]]<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>
| |
| *[[Infection]] with [[encapsulated microorganisms]] such as [[Streptococcus pneumonia]], [[Neisseria meningitides]] and [[Haemophilous influenzae]], and [[parasitic infections]] such as [[babesiosis]], [[malaria]].
| |
| *[[Waterhouse-Friedrichsen syndrome]] and [[Purpura fulminans]] <ref name="pmid27583208">{{cite journal| author=Hale AJ, LaSalvia M, Kirby JE, Kimball A, Baden R| title=Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult. | journal=IDCases | year= 2016 | volume= 6 | issue= | pages= 1-4 | pmid=27583208 | doi=10.1016/j.idcr.2016.08.004 | pmc=4995527 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27583208 }} </ref>
| |
| *[[Arterial thrombosis]]: includes [[coronary artery disease]] <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>
| |
| *[[Venous thrombosis]]: includes [[deep vein thrombosis]], [[pulmonary embolism]], [[splenic]] and [[portal vein thrombosis]]
| |
| *[[Pulmonary hypertension]], [[associated]] with [[right ventricular dysfunction]].
| |
|
| |
|
| ==Diagnosis== | | ==Diagnosis== |
| Line 114: |
Line 29: |
|
| |
|
| ==Treatment== | | ==Treatment== |
| [[Asplenia medical treatment|Medical Therapy]] | [[Asplenia surgical techniques|Surgery]] | [[Asplenia interventions|Interventions]] | [[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]] |
| ==Medical Therapy==
| |
| ===Emergency Medical Management of suspected sepsis in Asplenic patient===
| |
| Asplenia can cause [[sepsis]] and require immediate management:<ref name="pmid24855431">{{cite journal| author=Salvadori MI, Price VE, Canadian Paediatric Society, Infectious Diseases and Immunization Committee| title=Preventing and treating infections in children with asplenia or hyposplenia. | journal=Paediatr Child Health | year= 2014 | volume= 19 | issue= 5 | pages= 271-8 | pmid=24855431 | doi= | pmc=4029242 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24855431 }} </ref>
| |
| *Children with [[asplenia]] for every [[febrile illness]], must be seen by a physician immediately.
| |
| *[[Sepsis]] in individuals with [[asplenia]] or [[hyposplenia]] is a [[medical emergency]] as these [[patients]] can die within several hours of [[fever]] onset despite appearing well initially.
| |
| *[[Administration]] of [[antibiotic]] therapy should not be delayed and [[blood culture]] should be performed unless there is an obvious [[nonbacterial source]].
| |
| *[[Ceftriaxone]]: [[100 mg/kg/dose]], [[(maximum 2 g/dose)]] should be given in all [[asplenic patients]].
| |
| *[[Administer]] both [[ceftriaxone]] and [[vancomycin]] (60 mg/kg/day in divided doses every 6 h) in case of [[intermediate]] or [[high penicillin-resistant pneumococci]].
| |
| *If the [[patient]] is treated in a [[clinic]] or [[office setting]], refer [[immediately]] to the nearest [[emergency department]].
| |
| *Clinical [[deterioration]] can be rapid even after [[antibiotic administratin]] so changes in [[antibiotics]] should be done after [[culture reports]] available.
| |
| *[[Vancomycin]] and [[ciprofloxacin]] can be used if the patient has an [[allergy]] to [[penicillin]] or [[cephalosporin]]. Changes in [[antibiotics]] should be done after [[culture]] reports available.
| |
| *According to the [[Surviving]] [[Sepsis]] [[Campaign]] guidelines, to avoid poor outcomes, patients suspected of [[sepsis]] should be started on [[antibiotics]] within 1 hour and as per standard [[sepsis]] guidelines, [[aggressive]] [[intravenous (IV)]] [[hydration]] should also be promptly [[initiated]] as a part of [[supportive care]].<ref name="pmid28101605">{{cite journal| author=Rhodes A, Evans LE, Alhazzani W, Levy MM, Antonelli M, Ferrer R | display-authors=etal| title=Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock: 2016. | journal=Intensive Care Med | year= 2017 | volume= 43 | issue= 3 | pages= 304-377 | pmid=28101605 | doi=10.1007/s00134-017-4683-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28101605 }} </ref>
| |
| *Also, [[asplenic]] [[patients]] are prone to [[developing]] [[septic shock]], they may require [[vasopressors]] to maintain their [[blood pressure]] and if patients develop [[respiratory failure]], [[mechanical ventilation]] may be [[necessary]] for [[certain]] [[circumstances]].
| |
| | |
| ==Surgery==
| |
| The mainstay of [[treatment]] for [[asplenia]] is [[medical therapy]] and [[prevention]].<ref name="pmid11253134">{{cite journal| author=Waghorn DJ| title=Overwhelming infection in asplenic patients: current best practice preventive measures are not being followed. | journal=J Clin Pathol | year= 2001 | volume= 54 | issue= 3 | pages= 214-8 | pmid=11253134 | doi=10.1136/jcp.54.3.214 | pmc=1731383 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11253134 }} </ref>
| |
| | |
| ==Primary prevention==
| |
| ===Vaccination===
| |
| *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.
| |
| *[[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]].
| |
| *Apart from all these [[vaccines]], patients should be [[encouraged]] to [[receive]] [[influenza vaccine]], [[annual vaccination]] against the common [[strains]] of [[influenza]].<ref name="pmid26130882">{{cite journal| author=Huebner ML, Milota KA| title=Asplenia and fever. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 3 | pages= 340-1 | pmid=26130882 | doi=10.1080/08998280.2015.11929267 | pmc=4462215 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26130882 }} </ref>
| |
| | |
| ===Antibiotic Prophylaxis===
| |
| *Only [[Immunizations]] do not [[protect against]] [[infections]] with [[encapsulated bacteria]], [[antibiotic prophylaxis]] Should be given.<ref name="pmid24855431">{{cite journal| author=Salvadori MI, Price VE, Canadian Paediatric Society, Infectious Diseases and Immunization Committee| title=Preventing and treating infections in children with asplenia or hyposplenia. | journal=Paediatr Child Health | year= 2014 | volume= 19 | issue= 5 | pages= 271-8 | pmid=24855431 | doi= | pmc=4029242 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24855431 }} </ref>
| |
| **'''For children'''
| |
| ***All [[patients]] younger than [[five years]] of age should receive [[antibiotic prophylaxis]].
| |
| ***'''Birth to three months''': [[Escherichia coli]], [[Klebsiella]] are of concern in this [[age group]].
| |
| ****[[Amoxicillin]] or [[clavulanate]] 10 mg/kg/dose PO q12h, with [[penicillin VK]] 125 mg per dose PO q12h OR [[amoxicillin]] 10 mg/kg/dose q12h, as an [[alternative]] if not tolerated.
| |
| ***'''more than 3 months to five years'''
| |
| ****[[Penicillin]] VK 125 mg per dose PO q12h OR [[amoxicillin]] 10 mg/kg/dose PO q12h.
| |
| ****[[Liquid amoxicillin]] tastes better and may be [[better tolerated]] than [[liquid penicillin]].
| |
| ***'''more than 5 years'''
| |
| ****[[Penicillin V]] 250 mg or 300 mg per dose q12h OR [[amoxicillin]] 250 mg per dose q12h.
| |
| ****For [[penicillin]], 250 mg is a convenient dose for [[suspension]] but [[tablets]] are only available as 300 mg
| |
| *The [[infectious]] [[risk]] in [[asplenic]] patients is high during their [[entire life]] but it is highest during the first 2 years following [[splenectomy]] and the [[risk]] [[decreases]] over time.<ref name="pmid32787857">{{cite journal| author=Quéffélec C, Billet L, Duffau P, Lazaro E, Machelart I, Greib C | display-authors=etal| title=Prevention of infection in asplenic adult patients by general practitioners in France between 2013 and 2016 : Care for the asplenic patient in general practice. | journal=BMC Fam Pract | year= 2020 | volume= 21 | issue= 1 | pages= 163 | pmid=32787857 | doi=10.1186/s12875-020-01237-3 | pmc=7425533 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32787857 }} </ref>
| |
| *Along with [[vaccination]], [[antibiotic prophylaxis]] should be given.
| |
| *Long term [[prophylactic therapy]] [[oral antibiotic]] [[penicillin V]], or [[erythromycin]] in case of allergy, is required for at least [[2 years]] after [[splenectomy]] to cover the period during which the [[infectious]] risk is highest.
| |
| | |
| ===Malaria Prophylaxis===
| |
| *[[Asplenic]] and [[hyposplenic]] children must be advised of their [[increased]] [[risk]] of [[severe malaria]] and also take [[malaria prophylaxis]] as appropriate for their age and the type of [[malaria]] found in the area to which they are [[travelling]] and they should always [[seek]] [[travel advice]].<ref name="pmid19750611">{{cite journal| author=Committee to Advise on Tropical Medicine and Travel (CATMAT)| title=Canadian recommendations for the prevention and treatment of malaria among international travellers--2009. | journal=Can Commun Dis Rep | year= 2009 | volume= 35 Suppl 1 | issue= | pages= 1-82 | pmid=19750611 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19750611 }} </ref>
| |
| | |
| ==Secondary prevention==
| |
| Effective measures for the secondary prevention of asplenia include:
| |
| | |
| *[[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>
| |
| *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.
| |
| *The risk of [[infection]] can be significantly reduced by using [[systematic]], [[long-term approaches]] to care for [[asplenic patients]].
| |
| *[[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>
| |
| | |
| *
| |
|
| |
|
| ==Case Studies== | | ==Case Studies== |
| [[Asplenia case study one|Case #1]] | | [[Asplenia case study one|Case #1]] |
|
| |
| {{Hematology}}
| |
| {{Phakomatoses and other congenital malformations not elsewhere classified}}
| |
|
| |
|
| |
| [[de:Asplenie]]
| |
| [[nl:Asplenie]]
| |
| [[fi:Asplenia]]
| |
|
| |
|
| |
| | | |
| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}}
| |
|
| |
| [[Category:Medicine]] | | [[Category:Medicine]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| Line 188: |
Line 40: |
| [[Category:Immunology]] | | [[Category:Immunology]] |
| [[Category:Hematology]] | | [[Category:Hematology]] |
| <references />
| |