Aplastic anemia: Difference between revisions

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'''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]] .
{{SI}}
[[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]]
{{Infobox medical condition
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.
| name            = Aplastic anemia
| image          = [[File:13256_2010_Article_1435_Fig1_HTML.webp|alt=Bone marrow biopsy showing aplastic anemia]]
| caption        = Bone marrow biopsy showing aplastic anemia
| field          = [[Hematology]]
| symptoms        = [[Fatigue (medical)|Fatigue]], [[pallor]], [[shortness of breath]], [[frequent infections]], [[easy bruising]]
| complications  = [[Bleeding]], [[infection]], [[heart failure]]
| onset          = Any age, but more common in young adults and older adults
| duration        = Chronic
| causes          = [[Autoimmune disease]], [[chemotherapy]], [[radiation therapy]], [[toxic chemicals]], [[viral infections]]
| risks          = Exposure to [[benzene]], [[pesticides]], certain [[medications]]
| diagnosis      = [[Complete blood count]], [[bone marrow biopsy]]
| differential    = [[Myelodysplastic syndrome]], [[leukemia]], [[paroxysmal nocturnal hemoglobinuria]]
| treatment      = [[Immunosuppressive therapy]], [[bone marrow transplant]], [[blood transfusions]]
| prognosis      = Variable; depends on severity and response to treatment
| frequency      = Rare
}}
'''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.  


== '''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
Low numbers of platelets ([[thrmbocytopenia]]): May result in gum bleeding, nosebleeds or bleeding in the internal organs and skin bruises.
embed_source_url=http://www.youtube.com/v/w8-jx1dtg0U
Low  white blood cells (neutropenia): May present infections, recurrent infections, mouth sores.  
&rel=1
 
wrap = yes
100% of people have these symptoms
width=750
 
height=600
* Anemia(Low number of red blood cells or hemoglobin)
</youtube>
* [[Aplastic anemia]]
 
80%-99% of people have these symptoms
* [[Bone marrow]] hypocellularity (Bone marrow failure)
30%-79% of people have these symptoms
* [[Pancytopenia]] (Low blood cell count)
* [[Reticulocytopenia]]


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]].
==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]] 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==
==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.
 
==Treatment==
The following tests aid in determining differential diagnosis for aplastic anemia:
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]]").
#  Bone marrow aspirate and biopsy: to rule out other causes of pancytopenia (i.e. neoplastic infiltration or significant myelofibrosis).
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.
#  History of iatrogenic exposure to [[Cytotoxicity|cytotoxic]] chemotherapy: can cause transient bone marrow suppression
==Follow-up==
#  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)
Regular [[full blood count]]s are required to determine whether the patient is still in a state of remission.
#  Chest X-ray: infections
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.
#  Liver tests: liver diseases
== Gallery ==
#  Viral studies: viral infections
<gallery>
#  Vitamin B<sub>12</sub> and folate levels: vitamin deficiency
Symptoms_of_anemia.svg|Symptoms of anemia
#  Blood tests for [[paroxysmal nocturnal hemoglobinuria]]
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
#  Test for antibodies: immune competency
Give_Life_-_Donner_la_vie_(37346943046).jpg|Give Life - Donner la vie (37346943046)
 
</gallery>
== '''Treatment''' ==
==See also==
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:
*[[Fanconi anemia]]
* Blood transfusions to keep blood cells counts at acceptable levels
*[[Acquired pure red cell aplasia]]
* Blood and marrow stem cell transplants to replace damaged [[stem cells]] with health ones from a donor (another person)
==External links==
* Medications to stimulate the bone marrow, suppress the [[immune system]] , and prevent and treat infections
*[http://www.aamds.org Aplastic Anemia & MDS International Foundation]
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.
*[http://www.mayoclinic.com/health/aplastic-anemia/DS00322 Mayo Clinic]
Medications to stimulate the bone marrow, suppress the immune system , and prevent and treat infections
*[http://medic.med.uth.tmc.edu/ptnt/00001038.htm University of Texas]
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.
{{Stub}}
 
{{Medical resources
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.
|  ICD10          = {{ICD10|D|60||d|60}}-{{ICD10|D|61||d|60}}
 
|  ICD9          = {{ICD9|284}}
==New FDA approved medications==
|  OMIM          = 609135
The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.
|  MedlinePlus    = 000554
 
|  eMedicineSubj  = med
'''[[Eltrombopag]]''' '''(Brand name: [[Promacta]])'''
|  eMedicineTopic = 162
[[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.
|  DiseasesDB    = 866
 
|  MeshID        = D000741
== '''Prognosis''' ==
}}
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.
{{stub}}
{{rarediseases}}
{{Diseases of RBCs}}
{{Diseases of RBCs}}
{{DEFAULTSORT:Aplastic Anemia}}
[[Category:Aplastic anemias]]
[[Category:Aplastic anemias]]
[[Category:Autoimmune diseases]]
[[Category:Autoimmune diseases]]
[[Category:Hematopathology]]
[[Category:Hematopathology]]
[[Category:Blood disorders]]

Latest revision as of 00:51, 28 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Aplastic anemia
Bone marrow biopsy showing aplastic anemia
Synonyms N/A
Pronounce N/A
Specialty N/A
Symptoms Fatigue, pallor, shortness of breath, frequent infections, easy bruising
Complications Bleeding, infection, heart failure
Onset Any age, but more common in young adults and older adults
Duration Chronic
Types N/A
Causes Autoimmune disease, chemotherapy, radiation therapy, toxic chemicals, viral infections
Risks Exposure to benzene, pesticides, certain medications
Diagnosis Complete blood count, bone marrow biopsy
Differential diagnosis Myelodysplastic syndrome, leukemia, paroxysmal nocturnal hemoglobinuria
Prevention N/A
Treatment Immunosuppressive therapy, bone marrow transplant, blood transfusions
Medication N/A
Prognosis Variable; depends on severity and response to treatment
Frequency Rare
Deaths N/A


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[edit]

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[edit]

Diagnosis[edit]

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[edit]

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[edit]

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[edit]

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

See also[edit]

External links[edit]

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Diseases of red blood cells
↑
↓
Anemia
Nutritional
Hemolytic
(mostly normo-)
Hereditary
Acquired
Aplastic
(mostly normo-)
Blood tests
Other
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