Clinical Reference
Blood Disorder Information
Comprehensive clinical information authored by Dr. Suparno Chakrabarti and Dr. Mahak Agarwal.
Aplastic Anemia
Pediatric BMT — Dr. Suparno Chakrabarti
Aplastic Anemia in Children
The blood cells have a limited lifespan. Red blood cells survive 3 months, white cells survive for 24 hours and platelets survive for 5 days. The bone marrow is an amazing organ which produces billions of blood cells every day to keep up with the requirements of our body. When the bone marrow fails, the cell production slows down and often stops. This is called aplastic anemia.
The incidence of Aplastic anemia in Asian countries is 5 times higher than in Europe or USA.
Aplastic anemia results from damage to the mother cells or stem cells which are no longer able to produce normal blood cells. This can happen due to the following causes:
- Chemicals and toxins such as benzene and pesticides
- Infections such as hepatitis
- Drugs such as arsenic, gold, painkillers and some antibiotics
- Inherited conditions such as Fanconi's Anemia
- Most commonly, the cause is not known and thought to be due to an immune mediated attack by the body on its own stem cells.
The diagnosis of aplastic anemia needs awareness of the condition and a strong index of suspicion. The patient can present with fever due to infection, bleeding and bruising due to low platelet count and fatigue due to anemia.
A routine blood test shall show at least two of the three cell lines of red cells, white cells and platelets being low. A marrow biopsy is mandatory to confirm the diagnosis and rule out other causes of marrow failure like leukemia.
It is important to look for a causative factor and stop any drug or exposure to chemical that might have caused it.
The investigations include blood tests to look for infections, autoimmune diseases like lupus and chromosomal breakage study to rule out Fanconi's anemia.
Classification
- Severe: neutrophil count less than 500 cells/microliter (normal 1500-6000/microliter)
- Very Severe: neutrophil count less than 200 cells/microliter
- Nonsevere: none of the above
Supportive Therapy
We cannot survive without functioning blood cells. Immediate treatment involves transfusion of red cells and platelets and treatment of infections. However, the patient must be referred to a center where curative treatment can be given as soon as possible. Repeated blood transfusions reduce the success rate of BMT.
Immunosuppressive Treatment
Antithymocyte globulin, a drug produced from horse or rabbit by injecting human lymphocytes in them, is the main drug. It suppresses the immune system and allows the marrow to recover. This drug is given with another oral medicine called Cyclosporine. About 60-70% of the patients improve with this treatment. However, it takes 3-6 months for the marrow to recover and many patients may relapse or develop other marrow disorders in the future.
Blood and Marrow Transplantation (BMT)
BMT cures 90% of the patients with aplastic anemia if carried out early and from a matched family donor. BMT is the treatment of choice for young patients with a matched sibling donor.
For patients without a matched family donor, HAPLOIDENTICAL DONOR TRANSPLANTATION is now giving results as good as a matched family donor. At BLOODS-R-US, we offer the best survival for patients with aplastic anemia through BMT.
Adult BMT — Dr. Suparno Chakrabarti
Aplastic Anemia in Adults
The blood cells have a limited lifespan. Red blood cells survive 3 months, white cells survive for 24 hours and platelets survive for 5 days. The bone marrow is an amazing organ which produces billions of blood cells every day to keep up with the requirements of our body. When the bone marrow fails, the cell production slows down and often stops. This is called aplastic anemia.
The incidence of Aplastic anemia in Asian countries is 5 times higher than in Europe or USA.
Aplastic anemia results from damage to the mother cells or stem cells which are no longer able to produce normal blood cells. Causes include: Chemicals and toxins such as benzene and pesticides; Infections such as hepatitis; Drugs such as arsenic, gold, painkillers and some antibiotics; Inherited conditions such as Fanconi's Anemia. Most commonly, the cause is not known and thought to be due to an immune mediated attack by the body on its own stem cells.
Diagnosis
- The diagnosis of aplastic anemia needs awareness of the condition and a strong index of suspicion. The patient can present with fever, weakness or bleeding.
- A routine blood test shall show at least two of the three cell lines of red cells, white cells and platelets being low.
- A bone marrow examination is essential for confirmation of the diagnosis. It shows absence of normal blood cells and replacement with fat cells.
- It is important to look for a causative factor and stop any drug or exposure to chemical that might have caused the condition.
- The investigations include blood tests to look for infections, autoimmune diseases like lupus and chromosomal abnormalities.
Classification
- Severe: neutrophil count less than 500 cells/microliter (normal 1500-6000/microliter)
- Very Severe: neutrophil count less than 200 cells/microliter
- Nonsevere: none of the above
Supportive Therapy
We cannot survive without functioning blood cells. Immediate treatment involves transfusion of red cells and platelets. Infections have to be treated with high-end antibiotics.
Immunosuppressive Treatment
Antithymocyte globulin, a drug produced from horse or rabbit by injecting human lymphocytes in them, is the mainstay of treatment. This drug is given for 4 to 5 days and it takes 3 to 4 months for blood counts to recover. This treatment produces response in 60 to 70% of the patients.
Blood and Marrow Transplantation (BMT)
BMT cures 90% of the patients with aplastic anemia if carried out early and from a matched family donor. Other options like Haploidentical BMT or Unrelated donor BMT are also showing promising results.