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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 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]].<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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| *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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| *[[Overwhelming post-splenectomy infection]] (OPSI) occurs in [[5%]] of [[patients]] and has a [[mortality rate]] of [[38%–70%]].
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| *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>
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| ===Complications===
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| '''Common complications of asplenia include'''
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| *[[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>
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| *[[Infection]] with [[encapsulated microorganisms]] such as [[Streptococcus pneumonia]], [[Neisseria meningitides]] and [[Haemophilous influenzae]]
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| *[[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>
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| *[[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>
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| *[[Venous thrombosis]]: includes [[deep vein thrombosis]], [[pulmonary embolism]], [[splenic]] and [[portal vein thrombosis]]
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| *[[Pulmonary hypertension]], [[associated]] with [[right ventricular dysfunction]].
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| '''Less Common complications of asplenia include'''
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| *Patients with [[asplenia]] are also at risk for less common [[infections]] due to [[Capnocytophaga]], [[Babesia]], and [[malaria]].<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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| ===Prognosis===
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| *Prognosis of asplenia is [[poor]], if asplenic patients are not diagnosed on time, and do not receive proper [[vaccination]]. These patients are at [[high risk]] of [[infection]] leads to [[sepsis]], [[septic shock]], and [[death]].<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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| *Huebner and colleagues, in One case report provides evidence of the [[poor]] [[prognosis]] in [[asplenic]] patients who present with infection despite receiving standard medical care.<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>
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| *In [[Right isomerism]] (Ivemark syndrome) [[Prognosis]] is Poor, 80 % die within first year.<ref name="pmid22470785">{{cite journal| author=Agarwal H, Mittal SK, Kulkarni CD, Verma AK, Srivastava SK| title=Right isomerism with complex cardiac anomalies presenting with dysphagia--a case report. | journal=J Radiol Case Rep | year= 2011 | volume= 5 | issue= 4 | pages= 1-9 | pmid=22470785 | doi=10.3941/jrcr.v5i4.702 | pmc=3303439 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22470785 }} </ref>
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| ==Diagnosis== | | ==Diagnosis== |
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| ==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==
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| ===Emergency Medical Management of suspected sepsis in Asplenic patient===
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| 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>
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| *Children with [[asplenia]] for every [[febrile illness]], must be seen by a physician immediately.
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| *[[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.
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| *[[Administration]] of [[antibiotic]] therapy should not be delayed and [[blood culture]] should be performed unless there is an obvious [[nonbacterial source]].
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| *[[Ceftriaxone]]: [[100 mg/kg/dose]], [[(maximum 2 g/dose)]] should be given in all [[asplenic patients]].
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| *[[Administer]] both [[ceftriaxone]] and [[vancomycin]] (60 mg/kg/day in divided doses every 6 h) in case of [[intermediate]] or [[high penicillin-resistant pneumococci]].
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| *If the [[patient]] is treated in a [[clinic]] or [[office setting]], refer [[immediately]] to the nearest [[emergency department]].
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| *Clinical [[deterioration]] can be rapid even after [[antibiotic administratin]] so changes in [[antibiotics]] should be done after [[culture reports]] available.
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| *[[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.
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| *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>
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| *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]].
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| ==Surgery==
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| 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>
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| ==Primary prevention==
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| ===Vaccination===
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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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| *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>
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| ===Antibiotic Prophylaxis===
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| *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>
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| **'''For children'''
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| ***All [[patients]] younger than [[five years]] of age should receive [[antibiotic prophylaxis]].
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| ***'''Birth to three months''': [[Escherichia coli]], [[Klebsiella]] are of concern in this [[age group]].
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| ****[[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.
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| ***'''more than 3 months to five years'''
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| ****[[Penicillin]] VK 125 mg per dose PO q12h OR [[amoxicillin]] 10 mg/kg/dose PO q12h.
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| ****[[Liquid amoxicillin]] tastes better and may be [[better tolerated]] than [[liquid penicillin]].
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| ***'''more than 5 years'''
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| ****[[Penicillin V]] 250 mg or 300 mg per dose q12h OR [[amoxicillin]] 250 mg per dose q12h.
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| ****For [[penicillin]], 250 mg is a convenient dose for [[suspension]] but [[tablets]] are only available as 300 mg
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| *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>
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| *Along with [[vaccination]], [[antibiotic prophylaxis]] should be given.
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| *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.
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| ===Malaria Prophylaxis===
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| *[[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>
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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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