Aplastic anemia 196125 225369708 2008-07-13T09:31:42Z Jfdwolff 46555 /* Follow-up */ rephrase {{DiseaseDisorder infobox | Name = Aplastic anemia | ICD10 = {{ICD10|D|60||d|60}}-{{ICD10|D|61||d|60}} | ICD9 = {{ICD9|284}} | ICDO = | Image = | Caption = | OMIM = 609135 | OMIM_mult = | MedlinePlus = | eMedicineSubj = med | eMedicineTopic = 162 | DiseasesDB = 866 | MeshID = D000741 | }} '''Aplastic anemia''' is a condition where [[bone marrow]] does not produce sufficient new [[cell (biology)|cell]]s to replenish [[blood cells]]. The term 'aplastic' means the marrow suffers from an [[aplasia]] that renders it unable to function properly. [[Anemia]] is the condition of having reduced [[hemoglobin]] or red cell concentration in the blood. 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]]. ==Causes== One known cause is an [[autoimmune disorder]], where the [[white blood cell]]s attack the bone marrow. 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]], [[felbamate]], [[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. Aplastic anaemia is present in up to 2% of patients with acute [[viral hepatitis]]. ==Signs and symptoms== * [[Anemia]] with [[malaise]], [[pallor]] and associated symptoms * [[Thrombocytopenia]] (low platelet counts), leading to increased risk of [[hemorrhage]] and [[bruising]] * [[Leukopenia]] (low white blood cell count), leading to increased risk of [[infection]] ==Diagnosis== The condition needs to be differentiated from pure red cell aplasia. In aplastic anemia the patient has pancytopenia (i.e., anemia, neutropenia and thrombocytopenia) resulting in decrease of all formed elements. In contrast, pure red cell aplasia is characterized by reduction in red cells only. The diagnosis can only be confirmed on [[bone marrow examination]]. Before this procedure is undertaken, a patient will generally have had other [[blood test]]s to find diagnostic clues, including a [[complete blood count]] (CBC), [[renal function]] and [[electrolyte]]s, [[liver enzyme]]s, [[thyroid]] function tests, [[vitamin B12]] and [[folic acid]] levels. ==Treatment== Treating immune mediated 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.<ref name="pmid17229630">{{cite journal |author=Locasciulli A, Oneto R, Bacigalupo A, ''et al'' |title=Outcome of patients with acquired aplastic anemia given first line bone marrow transplantation or immunosuppressive treatment in the last decade: a report from the European Group for Blood and Marrow Transplantation (EBMT) |journal=Haematologica |volume=92 |issue=1 |pages=11–8 |year=2007 |pmid=17229630|doi=10.3324/haematol.10075}}</ref> 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]] (ALG) 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]].<ref name="titleNIH Clinical Center: Clinical Center News, NIH Clinical Center">{{cite web |url=http://www.cc.nih.gov/about/news/newsletter/2004/aug04/index.shtml |title=NIH Clinical Center: Clinical Center News, NIH Clinical Center |accessdate=2007-12-04 |format= |work=}}</ref> ==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.{{Fact|date=February 2007}} 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.{{Fact|date=February 2007}} 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 performed regularly in people with previous aplastic anemia to monitor for the development of PNH. ==See also== * [[Fanconi anemia]] * [[Acquired pure red cell aplasia]] ==References== {{reflist}} ==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] {{Hematology}} [[Category:Blood disorders]] [[Category:Autoimmune diseases]] [[bn:অবর্ধক রক্তশূন্যতা]] [[cs:Aplastická anémie]] [[de:Aplastische Anämie]] [[es:Anemia aplásica]] [[fr:Insuffisance médullaire]] [[it:Anemia aplastica]] [[he:אנמיה אפלסטית]] [[nl:Beenmergdepressie]] [[ja:再生不良性貧血]] [[pl:Niedokrwistość aplastyczna]] [[pt:Anemia aplástica]] [[ru:Апластическая анемия]] [[fi:Aplastinen anemia]] [[sv:Aplastisk anemi]] [[tr:Aplastik anemi]] [[zh:再生不良性貧血]]