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| | {| class="infobox" style="float:right;" |
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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''']] |
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| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{DiseaseDisorder infobox | | | {{DiseaseDisorder infobox | |
| {{Template:Aplastic anemia}}
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| Name = Aplastic anemia | | | Name = Aplastic anemia | |
| Image = Aplasticanemia.jpg | | | Image = Aplasticanemia.jpg | |
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| MeshID = D000741 | | | MeshID = D000741 | |
| }} | | }} |
| '''Editor-In-Chief:''' Aric Hall, M.D., Beth Israel Deaconess Medical Center, Boston, MA[mailto:achall@bidmc.harvard.edu]
| | {{Template:Aplastic anemia}} |
| | | {{CMG}}; {{AE}} {{N.F}}; Aric Hall, M.D., Beth Israel Deaconess Medical Center, Boston, MA[mailto:achall@bidmc.harvard.edu] |
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| ==Overview==
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| '''Aplastic anemia''' is a condition where [[bone marrow]] does not produce sufficient new [[cell (biology)|cell]]s to replenish [[blood]] cells.
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| The term 'aplastic' means the marrow suffers from an [[aplasia]] that renders it unable to function properly. [[Anemia]] is the condition of having fewer red blood cells than normal, or fewer than needed to function properly. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts of all three blood cell types: [[red blood cell]]s, [[white blood cell]]s, and [[platelets]].
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| ==Causes==
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| Aplastic anemia is a rare condition with incidence of two cases per million individuals per year in Western countries with rates two to three times as high in Asia.<ref name="pmid18391779">{{cite journal |author=Young NS, Scheinberg P, Calado RT |title=Aplastic anemia |journal=Curr. Opin. Hematol. |volume=15 |issue=3 |pages=162–8 |year=2008 |month=May |pmid=18391779 |doi=10.1097/MOH.0b013e3282fa7470 |url=}}</ref> This low incidence leads to difficulties in forming clear associations with causal factors.
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| One known cause is an [[autoimmune disorder]], where the [[white blood cell]]s attack the bone marrow.
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| In many cases, the [[etiology]] is impossible to determine, but aplastic anemia is sometimes associated with exposure to substances such as [[benzene]], [[radiation]], or to the use of certain drugs, including [[chloramphenicol]], [[carbamazepine]], [[phenytoin]], [[quinine]], and [[phenylbutazone]]. Many drugs are associated with aplasia mainly in the base of case reports but at a very low probability, As an example, chloramphenicol treatment is followed by aplasia in less than 1 in 40,000 treatment courses,and carbamazepine aplasia is even more rare.
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| Aplastic anaemia is present in up to 2% of patients with acute [[viral hepatitis]].
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| ==Signs and symptoms==
| | {{SK}} Aplastic anaemia, aplastic pancytopenia, bone marrow hypoplasia, hypoplastic bone marrow, bone marrow aplasia, aplastic bone marrow, Ehrlich anemia, Hypoplastic anemia; Refractory anemia |
| * [[Anemia]] with [[malaise]], [[pallor]] and associated symptoms
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| * [[Thrombocytopenia]] (low platelet counts), leading to increased risk of [[hemorrhage]] and [[bruising]]
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| * [[Leukopenia]] (low white blood cell count), leading to increased risk of [[infection]]
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| | ==[[Aplastic anemia overview|Overview]]== |
| | ==[[Aplastic anemia historical perspective|Historical Perspective]]== |
| | ==[[Aplastic anemia classification|Classification]]== |
| | ==[[Aplastic anemia pathophysiology|Pathophysiology]]== |
| | ==[[Aplastic anemia causes|Causes]]== |
| | ==[[Aplastic anemia differential diagnosis|Differentiating Aplastic anemia from other Diseases]]== |
| | ==[[Aplastic anemia epidemiology and demographics|Epidemiology and Demographics]]== |
| | ==[[Aplastic anemia risk factors|Risk Factors]]== |
| | ==[[Aplastic anemia screening|Screening]]== |
| | ==[[Aplastic_anemia_natural_history,_complications_and_prognosis|Natural History, Complications, and Prognosis]]== |
| ==Diagnosis== | | ==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.
| | [[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]] |
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| ==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]] |
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| 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]].
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| ==Prognosis==
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| 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.
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| Occasionally, milder cases of the disease resolve on their own. Relapses of previously controlled disease are, however, much more common.
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| 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.
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| 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%.
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| ==Follow-up== | |
| Regular [[full blood count]]s are required to determine whether the patient is still in a state of remission.
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| 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.
| | ==Related Chapters== |
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| ==See also== | |
| * [[Fanconi anemia]] | | * [[Fanconi anemia]] |
| * [[Acquired pure red cell aplasia]] | | * [[Acquired pure red cell aplasia]] |
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| ==External links==
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| * [http://www.aamds.org Aplastic Anemia & MDS International Foundation]
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| * [http://www.mayoclinic.com/health/aplastic-anemia/DS00322 Mayo Clinic]
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| * [http://medic.med.uth.tmc.edu/ptnt/00001038.htm University of Texas]
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| * {{MedlinePlusEncylopedia|000554}} -- Idiopathic aplastic anemia
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| * {{MedlinePlusEncylopedia|000529}} -- Secondary aplastic anemia
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| * [http://www.TheAAT.org.uk The Aplastic Anaemia Trust]
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| * [http://www.shannonstrust.org.uk/help-given.html Help for Aplastic Anemia sufferers]
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| ==References==
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| {{reflist|2}}
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| {{Hematology}}
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| [[bn:অবর্ধক রক্তশূন্যতা]]
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| [[de:Aplastische Anämie]]
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| [[es:Anemia aplásica]]
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| [[fr:Insuffisance médullaire]]
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| [[it:Anemia aplastica]]
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| [[nl:Beenmergdepressie]]
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| [[ja:再生不良性貧血]]
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| [[pt:Anemia aplástica]]
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| [[fi:Aplastinen anemia]]
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| [[sv:Aplastisk anemi]]
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| [[tr:Aplastik anemi]]
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| [[zh:再生不良性貧血]]
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| [[Category:Disease]] | | [[Category:Disease]] |
