Patient Education
What is Aplastic anemia?
Functional failure of the bone marrow giving rise to decreased production of all 3 cell lines namely red blood cells,white blood cells and platelets is known as Aplastic anemia.Due to decreased production of red blood cells the child cannot make adequate Hemoglobin causing decreased oxygenation to various organs in the body. Low white blood cells makes the child susceptible to infections and decreased platelets increases the chances of bleeding as the blood does not clot.
What causes Aplastic anemia?
The cause is idiopathic or unknown in most of the cases. However certain environmental toxins, radiation are also known to cause aplasia. Cancerous changes in the cells or certain immunological disorders can present with aplastic anemia as the primary manifestation. The causes include Infections such as hepatitis, Epstein-Barr virus (EBV), cytomegalovirus (CMV), parvovirus B19, or human immunodeficiency virus (HIV). History of drugs is important in aplastic anemia as certain medications can cause aplastic anemia in children. Last but not the least work up for autoimmune conditions should also be kept in mind in cases of aplastic anemia.
In every child diagnosed with aplastic anemia, it is imperative to rule out paroxysmal nocturnal hemoglobinuria or inherited genetic defects like Fanconi anemia,Dyskeratosis congenita,Shwachman-Diamond syndrome,Reticular dysgenesis,Amegakaryocytic thrombocytopenia and Familial aplastic anemias.
What are the symptoms of Aplastic anemia?
Due to low hemoglobin the child would suffer from symptoms of headache, dizziness, fatigue or lack of energy, shortness of breath, pallor, bleeding from various sites due to low platelets and fevers with repeated infections. The symptoms my mimic any other hematological disorder and the child would require further work up.
What tests are done to diagnose and confirm Aplastic anemia
Apart from history and physical examination, complete blood count and other blood tests, a bone marrow aspiration and biopsy( core biopsy) from the hip bones is a must to look at the marrow cellularity. Most of the times the marrow is replaced by fat spaces.
What is the treatment of aplastic anemia?
If a matched sibling donor is available these children should be offered upfront bone marrow transplant. In experienced centres, a matched unrelated and a haploidentical( half match) transplant is also feasible.
If transplant is not an option immunosuppressive therapy can be offered to these children but around 40-50% do not respond to this therapy. Supportive care in the form of blood and platelet transfusion is an essential component of aplastic anemia . Antibiotics at the time of infection and cotrimoxazole prophylaxis to prevent Pneumocystis carinii infection.
Things to avoid when diagnosed with Aplastic anemia
- No contact sports in view of low platelets.
- Avoid dental work in view of low white cell count.
- No travel to high altitudes in view of low hemoglobin.