Asplenia natural history, complications and prognosis
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.
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