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==Diagnosis==
==Diagnosis==
[[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]]
[[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]]").
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.
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==
==Related Chapters==
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==
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]]
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==External links==
==External links==
* [http://www.aamds.org Aplastic Anemia & MDS International Foundation]
* [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]
* [http://medic.med.uth.tmc.edu/ptnt/00001038.htm University of Texas]
* {{MedlinePlusEncylopedia|000554}} -- Idiopathic aplastic anemia
* {{MedlinePlusEncylopedia|000554}} -- Idiopathic aplastic anemia
* {{MedlinePlusEncylopedia|000529}} -- Secondary 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}}
{{Hematology}}

Revision as of 14:15, 21 September 2012

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Editor-In-Chief: Aric Hall, M.D., Beth Israel Deaconess Medical Center, Boston, MA[1]

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