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| [[File:Siren.gif|30px|link=Aplastic anemia resident survival guide]]|| <br> || <br>
| [[Aplastic anemia resident survival guide|'''Resident'''<br>'''Survival'''<br>'''Guide''']]
|}
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{DiseaseDisorder infobox |  
{{DiseaseDisorder infobox |  
   Name          = Aplastic anemia |
   Name          = Aplastic anemia |
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{{Template:Aplastic anemia}}  
{{Template:Aplastic anemia}}  
'''Editor-In-Chief:''' Aric Hall, M.D., Beth Israel Deaconess Medical Center, Boston, MA[mailto:achall@bidmc.harvard.edu]
{{CMG}}; {{AE}} {{N.F}}; Aric Hall, M.D., Beth Israel Deaconess Medical Center, Boston, MA[mailto:achall@bidmc.harvard.edu]
 
{{SK}} Aplastic anaemia, aplastic pancytopenia, bone marrow hypoplasia, hypoplastic bone marrow, bone marrow aplasia, aplastic bone marrow, Ehrlich anemia, Hypoplastic anemia; Refractory anemia


==[[Aplastic anemia overview|Overview]]==
==[[Aplastic anemia overview|Overview]]==
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==[[Aplastic_anemia_natural_history,_complications_and_prognosis|Natural History, Complications, and Prognosis]]==
==[[Aplastic_anemia_natural_history,_complications_and_prognosis|Natural History, Complications, and Prognosis]]==
==Diagnosis==
==Diagnosis==
 
[[Aplastic anemia diagnostic study of choice|Diagnostic Study of Choice]] | [[Aplastic anemia history and symptoms|History and Symptoms]] | [[Aplastic anemia physical examination|Physical Examination]] | [[Aplastic anemia laboratory findings|Laboratory Findings]] | [[Aplastic anemia electrocardiogram|Electrocardiogram]] | [[Aplastic anemia chest x ray|Chest X Ray]] | [[Aplastic anemia CT|CT]] | [[Aplastic anemia MRI|MRI]] | [[Aplastic anemia echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Aplastic anemia other imaging findings|Other Imaging Findings]] | [[Aplastic anemia other diagnostic studies|Other Diagnostic Studies]]
 
==Diagnosis==
The diagnosis can only be made on [[bone marrow examination]]. This will reveal a hypocellular bone marrow with fatty replacement of the normal hematopoetic precursors.  The primary diagnostic dilemma is differentiating aplastic anemia from a hypocellular [[myelodysplastic syndrome]]. Before this procedure is undertaken, a patient will generally have had other [[blood test]]s to find diagnostic clues, including a [[full blood count]], [[renal function]] and [[electrolyte]]s, [[liver enzyme]]s, [[thyroid]] function tests, [[vitamin B12]] and [[folic acid]] levels.


==Treatment==
==Treatment==
Treating aplastic anemia involves suppression of the [[immune system]], an effect achieved by daily [[medicine]] intake, or, in more severe cases, a [[bone marrow transplant]], a potential cure but a risky procedure. The transplanted bone marrow replaces the failing bone marrow cells with new ones from a matching donor. The [[pluripotency|pluripotent]] stem cells in the bone marrow reconstitute all three blood cell lines, giving the patient a new immune system, red blood cells, and platelets. However, besides the risk of graft failure, there is also a risk that the newly created white blood cells may attack the rest of the body ("[[graft-versus-host disease]]").
[[Aplastic anemia medical therapy|Medical Therapy]] | [[Aplastic anemia surgery|Surgery]] | [[Aplastic anemia primary prevention|Primary Prevention]] | [[Aplastic anemia secondary prevention|Secondary Prevention]] | [[Aplastic anemia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Aplastic anemia future or investigational therapies|Future or Investigational Therapies]]
 
==Case Studies==
Medical therapy of aplastic anemia often includes a short course of [[anti-thymocyte globulin]] (ATG or [[anti-lymphocyte globulin]]) and several months of treatment with [[cyclosporin]] to modulate the [[immune system]]. Mild [[chemotherapy]] with agents such as [[cyclophosphamide]] and [[vincristine]] may also be effective. [[Antibody|Antibodies]] therapy, such as ATG, targets T-cells, which are believed to attack the bone marrow. [[Steroids]] are generally ineffective.
[[Aplastic anemia case study one|Case #1]]
 
In the past, before the above treatments became available, patients with low leukocyte counts were often confined to a sterile room or bubble (to reduce risk of infections), as in the famed case of [[Ted DeVita]].
 
==Prognosis==
Untreated aplastic anemia is an illness that leads to rapid death, typically within six months.  If the disease is diagnosed correctly and initial treatment is begun promptly, then the survival rate for the next five to ten years is substantially improved, and many patients live well beyond that length of time.
 
Occasionally, milder cases of the disease resolve on their own.  Relapses of previously controlled disease are, however, 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%.


==Follow-up==
==Related Chapters==
Regular [[full blood count]]s are required to determine whether the patient is still in a state of remission.
 
10-33% of all patients develop the [[rare disease]] [[paroxysmal nocturnal hemoglobinuria]] (PNH, anemia with thrombopenia and/or [[thrombosis]]), which has been explained as an escape mechanism by the bone marrow against destruction by the immune system. [[Flow cytometry]] testing is probably warranted in all PNH patients with recurrent aplasia.
 
==See also==
* [[Fanconi anemia]]
* [[Fanconi anemia]]
* [[Acquired pure red cell aplasia]]
* [[Acquired pure red cell aplasia]]
==External links==
* [http://www.aamds.org Aplastic Anemia & MDS International Foundation]
* [http://www.mayoclinic.com/health/aplastic-anemia/DS00322 Mayo Clinic]
* [http://medic.med.uth.tmc.edu/ptnt/00001038.htm University of Texas]
* {{MedlinePlusEncylopedia|000554}} -- Idiopathic aplastic anemia
* {{MedlinePlusEncylopedia|000529}} -- Secondary aplastic anemia
* [http://www.TheAAT.org.uk The Aplastic Anaemia Trust]
* [http://www.shannonstrust.org.uk/help-given.html Help for Aplastic Anemia sufferers]
==References==
{{reflist|2}}
{{Hematology}}
[[bn:অবর্ধক রক্তশূন্যতা]]
[[de:Aplastische Anämie]]
[[es:Anemia aplásica]]
[[fr:Insuffisance médullaire]]
[[it:Anemia aplastica]]
[[nl:Beenmergdepressie]]
[[ja:再生不良性貧血]]
[[pt:Anemia aplástica]]
[[fi:Aplastinen anemia]]
[[sv:Aplastisk anemi]]
[[tr:Aplastik anemi]]
[[zh:再生不良性貧血]]
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[[Category:Disease]]
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Latest revision as of 16:54, 19 October 2018



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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nazia Fuad M.D.; Aric Hall, M.D., Beth Israel Deaconess Medical Center, Boston, MA[2]

Synonyms and keywords: Aplastic anaemia, aplastic pancytopenia, bone marrow hypoplasia, hypoplastic bone marrow, bone marrow aplasia, aplastic bone marrow, Ehrlich anemia, Hypoplastic anemia; Refractory anemia

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Aplastic anemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters