Asplenia natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
If left [[untreated]], | If left [[untreated]], patients with [[asplenia]] or [[hyposplenia]] are at risk of [[life-threatening]] [[infection]]. Common complications including [[overwhelming post-splenectomy infection (OPSI)]], [[Infection]] with [[encapsulated microorganisms]] such as [[Streptococcus pneumonia]], [[Neisseria meningitides]] and [[Haemophilous influenzae]], arterial and [[Venous thromboembolism|venous thrombosis]], [[Waterhouse-Friedrichsen syndrome]]. Less common complications include [[infections]] due to [[Capnocytophaga]], [[Babesia]], and [[malaria]]. Prognosis of asplenia is poor. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
*If left [[untreated]], | |||
*Patients with [[functional asplenia]] and [[hyposplenia]] who have not [[undergone]] a [[splenectomy]] can present with a [[life-threatening]] [[infection]] | *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> | ||
*Patients with [[functional asplenia]] and [[hyposplenia]] who have not [[undergone]] a [[splenectomy]] can present with a [[life-threatening]] [[infection]] comparable to an [[OPSI]]. | |||
*[[Overwhelming post-splenectomy infection]] (OPSI) occurs in [[5%]] of [[patients]] and has a [[mortality rate]] of [[38%–70%]]. | *[[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 | *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> | ||
===Complications=== | ===Complications=== | ||
'''Common complications''' | '''Common complications''' | ||
*Recurrent infections<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> | *Recurrent infections<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]] | *[[Infection]] with [[encapsulated microorganisms]] such as [[Streptococcus pneumonia]], [[Neisseria meningitides]] and [[Haemophilous influenzae]] | ||
| Line 24: | Line 26: | ||
'''Less Common complications''' | '''Less Common complications''' | ||
*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> | *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> | ||
===Prognosis=== | ===Prognosis=== | ||
*Prognosis of asplenia is | |||
*Huebner and colleagues, in One case report provides evidence of the | *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> | ||
*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> | |||
*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> | *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> | ||
Latest revision as of 04:57, 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
If left untreated, patients with asplenia or hyposplenia are at risk of life-threatening infection. Common complications including overwhelming post-splenectomy infection (OPSI), Infection with encapsulated microorganisms such as Streptococcus pneumonia, Neisseria meningitides and Haemophilous influenzae, arterial and venous thrombosis, Waterhouse-Friedrichsen syndrome. Less common complications include infections due to Capnocytophaga, Babesia, and malaria. Prognosis of asplenia is poor.
Natural History, Complications, and Prognosis
Natural History
- If left untreated, patients with asplenia or hyposplenia are at risk of life-threatening infection.[1]
- Patients with functional asplenia and hyposplenia who have not undergone a splenectomy can present with a life-threatening infection comparable to an OPSI.
- 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.[2]
Complications
Common complications
- Recurrent infections[1]
- Infection with encapsulated microorganisms such as Streptococcus pneumonia, Neisseria meningitides and Haemophilous influenzae
- Waterhouse-Friedrichsen syndrome and Purpura fulminans [3]
- Arterial thrombosis and coronary artery disease [2]
- Venous thrombosis such as deep vein thrombosis, pulmonary embolism, splenic and portal vein thrombosis
- Pulmonary hypertension, associated with right ventricular dysfunction.
Less Common complications
- Patients with asplenia are also at risk for less common infections due to Capnocytophaga, Babesia, and malaria.[4]
Prognosis
- 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.[1]
- 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.[5]
- In Right isomerism (Ivemark syndrome) Prognosis is Poor, 80 % die within first year.[6]
References
- ↑ 1.0 1.1 1.2 Kirkineska L, Perifanis V, Vasiliadis T (2014). "Functional hyposplenism". Hippokratia. 18 (1): 7–11. PMC 4103047. PMID 25125944.
- ↑ 2.0 2.1 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). - ↑ Hale AJ, LaSalvia M, Kirby JE, Kimball A, Baden R (2016). "Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult". IDCases. 6: 1–4. doi:10.1016/j.idcr.2016.08.004. PMC 4995527. PMID 27583208.
- ↑ Lee GM (2020). "Preventing infections in children and adults with asplenia". Hematology Am Soc Hematol Educ Program. 2020 (1): 328–335. doi:10.1182/hematology.2020000117. PMC 7727556 Check
|pmc=value (help). PMID 33275684 Check|pmid=value (help). - ↑ Huebner ML, Milota KA (2015). "Asplenia and fever". Proc (Bayl Univ Med Cent). 28 (3): 340–1. doi:10.1080/08998280.2015.11929267. PMC 4462215. PMID 26130882.
- ↑ Agarwal H, Mittal SK, Kulkarni CD, Verma AK, Srivastava SK (2011). "Right isomerism with complex cardiac anomalies presenting with dysphagia--a case report". J Radiol Case Rep. 5 (4): 1–9. doi:10.3941/jrcr.v5i4.702. PMC 3303439. PMID 22470785.