Aplastic anemia natural history, complications and prognosis: Difference between revisions
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{{Aplastic anemia}} | {{Aplastic anemia}} | ||
'''Editor(s)-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]] [mailto:charlesmichaelgibson@gmail.com] | '''Editor(s)-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]] [mailto:charlesmichaelgibson@gmail.com]; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.D.]] [mailto:psingh13579@gmail.com] {{N.F}} | ||
==Overview== | ==Overview== | ||
Aplastic anaemia has a mixed clinical course. Untreated aplastic anemia leads to rapid death, typically within six months. Complications following development of aplastic anemia are infections, bleeding and [[paroxysmal nocturnal hemoglobinuria]]. Graft-versus-host disease and graft failure are the possible complications following bone marrow transplant in these patients. Mild cases resolve on their own requiring little or no treatment. Well-matched bone marrow transplants from siblings have been successful in young individuals with a long-term survival rate of 80%-90%. | |||
==Natural History== | ==Natural History== | ||
Untreated aplastic anemia | * Aplastic anaemia has a mixed clinical course. <ref name="pmid20524094">{{cite journal |vauthors=Kwon JH, Kim I, Lee YG, Koh Y, Park HC, Song EY, Kim HK, Yoon SS, Lee DS, Park SS, Shin HY, Park S, Park MH, Ahn HS, Kim BK |title=Clinical course of non-severe aplastic anemia in adults |journal=Int. J. Hematol. |volume=91 |issue=5 |pages=770–5 |date=June 2010 |pmid=20524094 |doi=10.1007/s12185-010-0601-1 |url=}}</ref> | ||
* Some patients develop mild symptoms that require little or no therapy. | |||
* Others develop life-threatening pancytopenia presenting with a medical emergency. | |||
* Untreated aplastic anemia leads to rapid death, typically within six months. | |||
==Complications== | ==Complications== | ||
Aplastic anemia patients can develop following complications:<ref name="pmid24396488">{{cite journal |vauthors=Guo D, Liu Q, Li B, Teng Q |title=Severe aplastic anemia preceding acute monocytic leukemia in an adult with acquired trisomy 21: A case report |journal=Oncol Lett |volume=7 |issue=2 |pages=565–567 |date=February 2014 |pmid=24396488 |pmc=3881932 |doi=10.3892/ol.2013.1724 |url=}}</ref> | |||
* | * Infections | ||
* Bleeding | |||
* [[paroxysmal nocturnal hemoglobinuria]] (PNH, anemia with thrombopenia and/or [[thrombosis]]) | |||
* Graft-versus-host disease | |||
* Graft failure. | |||
* Myelodysplastic syndrome | |||
==Prognosis== | ==Prognosis== | ||
* Correct and prompt diagnosis with early | * Correct and prompt diagnosis with early therapy improves the 5 year survival rate.<ref name="pmid21495931">{{cite journal |vauthors=Dezern AE, Brodsky RA |title=Clinical management of aplastic anemia |journal=Expert Rev Hematol |volume=4 |issue=2 |pages=221–30 |date=April 2011 |pmid=21495931 |pmc=3138728 |doi=10.1586/ehm.11.11 |url=}}</ref> | ||
* Occasionally, milder cases of the disease resolve on their own. | * Occasionally, milder cases of the disease resolve on their own. | ||
* Relapses of previously controlled disease are | * Relapses of previously controlled disease are, much more common. | ||
* Well-matched bone marrow transplants from siblings have been successful in young, otherwise healthy people, with a long-term survival rate of 80%-90%. | * Well-matched bone marrow transplants from siblings have been successful in young, otherwise healthy people, with a long-term survival rate of 80%-90%. | ||
* Most successful BMT recipients eventually reach a point where they consider themselves cured for all practical purposes, although they need to be compliant with follow-up care permanently. | * Most successful BMT recipients eventually reach a point where they consider themselves cured for all practical purposes, although they need to be compliant with follow-up care permanently. | ||
Latest revision as of 18:34, 20 August 2018
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Editor(s)-in-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.D. [2] Nazia Fuad M.D.
Overview
Aplastic anaemia has a mixed clinical course. Untreated aplastic anemia leads to rapid death, typically within six months. Complications following development of aplastic anemia are infections, bleeding and paroxysmal nocturnal hemoglobinuria. Graft-versus-host disease and graft failure are the possible complications following bone marrow transplant in these patients. Mild cases resolve on their own requiring little or no treatment. Well-matched bone marrow transplants from siblings have been successful in young individuals with a long-term survival rate of 80%-90%.
Natural History
- Aplastic anaemia has a mixed clinical course. [1]
- Some patients develop mild symptoms that require little or no therapy.
- Others develop life-threatening pancytopenia presenting with a medical emergency.
- Untreated aplastic anemia leads to rapid death, typically within six months.
Complications
Aplastic anemia patients can develop following complications:[2]
- Infections
- Bleeding
- paroxysmal nocturnal hemoglobinuria (PNH, anemia with thrombopenia and/or thrombosis)
- Graft-versus-host disease
- Graft failure.
- Myelodysplastic syndrome
Prognosis
- Correct and prompt diagnosis with early therapy improves the 5 year survival rate.[3]
- Occasionally, milder cases of the disease resolve on their own.
- Relapses of previously controlled disease are, much more common.
- Well-matched bone marrow transplants from siblings have been successful in young, otherwise healthy people, with a long-term survival rate of 80%-90%.
- Most successful BMT recipients eventually reach a point where they consider themselves cured for all practical purposes, although they need to be compliant with follow-up care permanently.
- Older people (who are generally too frail to undergo bone marrow transplants) and people who are unable to find a good bone marrow match have five year survival rate of up to 75%.
References
- ↑ Kwon JH, Kim I, Lee YG, Koh Y, Park HC, Song EY, Kim HK, Yoon SS, Lee DS, Park SS, Shin HY, Park S, Park MH, Ahn HS, Kim BK (June 2010). "Clinical course of non-severe aplastic anemia in adults". Int. J. Hematol. 91 (5): 770–5. doi:10.1007/s12185-010-0601-1. PMID 20524094.
- ↑ Guo D, Liu Q, Li B, Teng Q (February 2014). "Severe aplastic anemia preceding acute monocytic leukemia in an adult with acquired trisomy 21: A case report". Oncol Lett. 7 (2): 565–567. doi:10.3892/ol.2013.1724. PMC 3881932. PMID 24396488.
- ↑ Dezern AE, Brodsky RA (April 2011). "Clinical management of aplastic anemia". Expert Rev Hematol. 4 (2): 221–30. doi:10.1586/ehm.11.11. PMC 3138728. PMID 21495931.