Aplastic anemia: Difference between revisions

Revision as of 12:38, 1 March 2025

Aplastic anemia is a condition where the bone marrow does not produce enough, or any, new cells to replenish the blood cells.

The term 'aplastic' refers to the inability of the marrow to function properly. Anemia is the condition of having fewer 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 on all three blood cell types: red blood cells, white blood cells, and platelets.

Causes

One known cause is an autoimmune disorder, where the white blood cells 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 and phenylbutazone.

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 diagnosis can only be made on bone marrow biopsy. Before this procedure is undertaken, a patient will generally have had other blood tests to find diagnostic clues, including a full blood count, renal function and electrolytes, liver enzymes, thyroid function tests, vitamin B12 and folic acid levels.

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 curing but risky procedure. Bone marrow transplant replaces the old bone marrow cells with new ones from a donor, giving the patient a new immune system. There is 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 ciclosporin to modulate the immune system. Mild chemotherapy with agents such as cyclophosphamide and vincristine may also be effective. Antibodies therapy, such as ATG, targets T-cells, which are believed to attack the bone marrow. Steroids are generally ineffective.

Follow-up

Regular full blood counts 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.

Gallery

Symptoms of anemia
Air Station Elizabeth City invites Braeden Hahn for tour 150706-G-DN496-301
Give Life - Donner la vie (37346943046)

External links

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@@ Line 1: / Line 1: @@
-'''Aplastic Anemia''' is a rare disease in which the [[bone marrow]] stops producing enough [[Blood cell|blood cells]]. Bone marrow is a sponge-like tissue inside the bones that makes [[stem cells]] that develop into [[red blood cells]],[[white blood cells]], and [[platelets]] .
+[[File:13256 2010 Article 1435 Fig1 HTML.webp|alt=Hypocellular bone marrow|thumb|Hypocellular bone marrow showed only a little hematopoesis and many fat cells]][[File:Give Life - Donner la vie (37394425121).jpg|thumb|Bone marrow transplant recipient]]
-[[File:13256 2010 Article 1435 Fig1 HTML.webp|alt=Hypocellular bone marrow|thumb|Hypocellular bone marrow showed only a little hematopoesis and many fat cells]]
+'''Aplastic anemia''' is a condition where the [[bone marrow]] does not produce enough, or any, new [[cell (biology)|cell]]s to replenish the [[blood]] cells.
-There are fewer than 20,000 cases a year in the US. Aplastic Anemia can be treated by a medical professional. The blood disease is not always deadly, but can be after lots of bleeding both internal and external.
-== '''Symptoms''' ==
+<youtube>
-The symptoms of aplastic anemia vary depending on how severe it is and how low blood counts are. Signs and symptoms may include:
+title='''{{PAGENAME}}'''
-Low numbers of [[red blood cells]] (anemia): May cause paleness (pallor), headache, palpitations, rapid heart rate, feeling out of breath, fatigue, or foot swelling.
+movie_url=http://www.youtube.com/v/w8-jx1dtg0U
+&rel=1
+embed_source_url=http://www.youtube.com/v/w8-jx1dtg0U
+&rel=1
+wrap = yes
+width=750
+height=600
+</youtube>
-Low numbers of platelets ([[thrmbocytopenia]]): May result in gum bleeding, nosebleeds or bleeding in the internal organs and skin bruises.
+The term 'aplastic' refers to the inability of the marrow to function properly.  [[Anemia]] is the condition of having fewer 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 on all three blood cell types:  [[red blood cell]]s, [[white blood cell]]s, and [[platelets]].
-Low  white blood cells (neutropenia): May present infections, recurrent infections, mouth sores.
-% of people have these symptoms
+==Causes==
+One known cause is an [[autoimmune disorder]], where the [[white blood cell]]s attack the bone marrow.
-* Anemia(Low number of red blood cells or hemoglobin)
+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]] and [[phenylbutazone]].
-* [[Aplastic anemia]]
-%-99% of people have these symptoms
+==Signs and symptoms==
-* [[Bone marrow]] hypocellularity (Bone marrow failure)
-%-79% of people have these symptoms
+*[[Anemia]] with [[malaise]], [[pallor]] and associated symptoms
-* [[Pancytopenia]] (Low blood cell count)
+*[[Thrombocytopenia]] (low platelet counts), leading to increased risk of [[hemorrhage]] and [[bruising]]
-* [[Reticulocytopenia]]
+*[[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., leukopenia 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]], [[renal function]] and [[electrolyte]]s, [[liver enzyme]]s, [[thyroid]] function tests, [[vitamin B12|vitamin B<sub>12</sub>]] and [[folic acid]] levels.
+The diagnosis can only be made on [[bone marrow biopsy]]. 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.
-The following tests aid in determining differential diagnosis for aplastic anemia:
+==Treatment==
-#  Bone marrow aspirate and biopsy: to rule out other causes of pancytopenia (i.e. neoplastic infiltration or significant myelofibrosis).
+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 curing but risky procedure. Bone marrow transplant replaces the old bone marrow cells with new ones from a donor, giving the patient a new immune system. There is a risk that the newly created white blood cells may attack the rest of the body ("[[graft-versus-host disease]]").
-#  History of iatrogenic exposure to [[Cytotoxicity|cytotoxic]] chemotherapy: can cause transient bone marrow suppression
-#  X-rays, computed tomography (CT) scans, or ultrasound imaging tests: enlarged lymph nodes (sign of lymphoma), kidneys and bones in arms and hands (abnormal in Fanconi anemia)
-#  Chest X-ray: infections
-#  Liver tests: liver diseases
-#  Viral studies: viral infections
-#  Vitamin B<sub>12</sub> and folate levels: vitamin deficiency
-#  Blood tests for [[paroxysmal nocturnal hemoglobinuria]]
-#  Test for antibodies: immune competency
-== '''Treatment''' ==
+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 [[ciclosporin]] 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.
-Treatment for aplastic anemia varies depending on the severity. While some individuals with mild to moderate aplastic anemia may not require treatment, for others, treatment may include:
-* Blood transfusions to keep blood cells counts at acceptable levels
-* Blood and marrow stem cell transplants to replace damaged [[stem cells]]  with health ones from a donor (another person)
-* Medications to stimulate the bone marrow, suppress the [[immune system]] , and prevent and treat infections
-Blood and marrow [[stem cell transplant]] may cure aplastic anemia in some instances. This treatment option works best in children and young adults with severe aplastic anemia who are otherwise in good health.
-Medications to stimulate the bone marrow, suppress the immune system , and prevent and treat infections
-For patients with severe aplastic anemia who are under the age of 20 years, and those ages 20 to 50 years who are otherwise in good health the first option is the transplant when a sibling donor is available. For those who do not have an available sibling donor, the medication eltrombopag or eltrombopag plus immunesuppression therapy can be used.
-For patients over 50 years of age, the decision is based on the patient’s overall health, and preferences, and treatment may include eltrombopag or eltrombopag plus immunesuppression therapy (horse anti-thymocyte globulin (ATG), cyclosporin A (CSA), and glucocorticoids). People older than 50 years old have more risks of having rejection with the transplant and have greater risks of treatment toxicity and early mortality.
+==Follow-up==
+Regular [[full blood count]]s are required to determine whether the patient is still in a state of remission.
-==New FDA approved medications==
+-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.
-The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.
-'''[[Eltrombopag]]''' '''(Brand name: [[Promacta]])'''
+== Gallery ==
-[[Eltrombopag]] (Promacta) was approved in combination with standard immunosuppressive therapy for the first-line treatment of adult and pediatric patients 2 years and older with severe aplastic anemia.
+<gallery>
+Symptoms_of_anemia.svg|Symptoms of anemia
+Air_Station_Elizabeth_City_invites_Braeden_Hahn_for_tour_150706-G-DN496-301.jpg|Air Station Elizabeth City invites Braeden Hahn for tour 150706-G-DN496-301
+Give_Life_-_Donner_la_vie_(37346943046).jpg|Give Life - Donner la vie (37346943046)
+</gallery>
+==See also==
+*[[Fanconi anemia]]
+*[[Acquired pure red cell aplasia]]
-== '''Prognosis''' ==
+==External links==
-A small number of people with aplastic anemia may spontaneously recover with supportive care; however, for most individuals, the condition worsens without identification and treatment of the underlying cause and/or treatment of the disease. [[Bone marrow transplantation]] may cure the disease in children and young patients and has a 10 year survival rate of approximately 73%. For many, bone marrow transplant is not an option due to the risks and potential long-term side effects.
+*[http://www.aamds.org Aplastic Anemia & MDS International Foundation]
-{{stub}}
+*[http://www.mayoclinic.com/health/aplastic-anemia/DS00322 Mayo Clinic]
-{{rarediseases}}
+*[http://medic.med.uth.tmc.edu/ptnt/00001038.htm University of Texas]
+{{Stub}}
+{{Medical resources
+|  ICD10          = {{ICD10|D|60||d|60}}-{{ICD10|D|61||d|60}}
+|  ICD9           = {{ICD9|284}}
+|  OMIM           = 609135
+|  MedlinePlus    = 000554
+|  eMedicineSubj  = med
+|  eMedicineTopic = 162
+|  DiseasesDB     = 866
+|  MeshID         = D000741
+}}
 {{Diseases of RBCs}}
+{{DEFAULTSORT:Aplastic Anemia}}
 [[Category:Aplastic anemias]]
 [[Category:Autoimmune diseases]]
 [[Category:Hematopathology]]
-[[Category:Blood disorders]]