Evans syndrome natural history, complications and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Evans syndrome}} | {{Evans syndrome}} | ||
{{CMG}} {{AE}}{{Faizan}} | {{CMG}} {{AE}}{{Faizan}} | ||
==Overview== | ==Overview== | ||
If left untreated, patients with Evans syndrome have periods of exacerbation. Common complications of Evans syndrome include thrombocytopenia and autoimmune haemolytic anaemia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. | |||
==Natural History== | ==Natural History== | ||
* The disease has a chronic course with periods of exacerbation and remissions. The remissions are induced by treatment. | * The disease has a chronic course with periods of exacerbation and remissions. The remissions are induced by treatment. | ||
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* In more serious cases it can massively reduce life span and is seriously life threatening. | * In more serious cases it can massively reduce life span and is seriously life threatening. | ||
* Evan's Syndrome is rare and has a reported [[mortality rate]] of just under 18%. | * Evan's Syndrome is rare and has a reported [[mortality rate]] of just under 18%. | ||
* It has been observed that there is a risk of developing other autoimmune problems and [[hypogammaglobulinemia]],<ref name="pmid3071168">{{cite journal |author=Wang WC |title=Evans syndrome in childhood: pathophysiology, clinical course, and treatment |journal=The American journal of pediatric hematology/oncology |volume=10 |issue=4 |pages=330–8 |year=1988 |pmid=3071168 |doi=10.1097/00043426-198824000-00013}}</ref> with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- [[T cells]] which is a strong predictor for having [[autoimmune lymphoproliferative syndrome]].<ref name="pmid15542578">{{cite journal |author=Teachey DT |title=Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS) |journal=Blood |volume=105 |issue=6 |pages=2443–8 |year=2005 |pmid=15542578 |doi=10.1182/blood-2004-09-3542 |url=http://bloodjournal.hematologylibrary.org/cgi/content/full/105/6/2443 |author2=Manno CS |author3=Axsom KM |display-authors=3 |last4=Andrews |first4=T |last5=Choi |first5=JK |last6=Greenbaum |first6=BH |last7=McMann |first7=JM |last8=Sullivan |first8=KE |last9=Travis |first9=SF}}</ref> | * It has been observed that there is a risk of developing other autoimmune problems and [[hypogammaglobulinemia]],<ref name="pmid3071168">{{cite journal |author=Wang WC |title=Evans syndrome in childhood: pathophysiology, clinical course, and treatment |journal=The American journal of pediatric hematology/oncology |volume=10 |issue=4 |pages=330–8 |year=1988 |pmid=3071168 |doi=10.1097/00043426-198824000-00013}}</ref> with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- [[T cells]] which is a strong predictor for having [[autoimmune lymphoproliferative syndrome]].<ref name="pmid15542578">{{cite journal |author=Teachey DT |title=Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS) |journal=Blood |volume=105 |issue=6 |pages=2443–8 |year=2005 |pmid=15542578 |doi=10.1182/blood-2004-09-3542 |url=http://bloodjournal.hematologylibrary.org/cgi/content/full/105/6/2443 |author2=Manno CS |author3=Axsom KM |display-authors=3 |last4=Andrews |first4=T |last5=Choi |first5=JK |last6=Greenbaum |first6=BH |last7=McMann |first7=JM |last8=Sullivan |first8=KE |last9=Travis |first9=SF}}</ref> | ||
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{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
Revision as of 16:43, 12 January 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2]
Overview
If left untreated, patients with Evans syndrome have periods of exacerbation. Common complications of Evans syndrome include thrombocytopenia and autoimmune haemolytic anaemia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.
Natural History
- The disease has a chronic course with periods of exacerbation and remissions. The remissions are induced by treatment.
Complications
- Thrombocytopenia
- Coombs positive, direct
- Autoimmune haemolytic anaemia
Prognosis
- Given the fact that the signs and symptoms of this disease are related directly to the abnormal laboratory findings, the prognosis depends on the patient’s response to treatment. Spontaneous remissions of each of the individual component conditions have been reported. If the child responds well to the treatment and the levels of platelets and red blood cells increase, the child can expect to live a normal life. Medications will be needed life long, and laboratory tests will need to be constantly monitored to detect any abnormal changes so that treatment can be adjusted.
- In more serious cases it can massively reduce life span and is seriously life threatening.
- Evan's Syndrome is rare and has a reported mortality rate of just under 18%.
- It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia,[1] with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- T cells which is a strong predictor for having autoimmune lymphoproliferative syndrome.[2]
References
- ↑ Wang WC (1988). "Evans syndrome in childhood: pathophysiology, clinical course, and treatment". The American journal of pediatric hematology/oncology. 10 (4): 330–8. doi:10.1097/00043426-198824000-00013. PMID 3071168.
- ↑ Teachey DT; Manno CS; Axsom KM; et al. (2005). "Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS)". Blood. 105 (6): 2443–8. doi:10.1182/blood-2004-09-3542. PMID 15542578.