Aplastic Anemia

Aplastic anemia is a condition which arises when the body stops producing enough new blood cells, or in other wordsit is a blood disorder in which the bone marrow (the soft tissue in the center of bones) doesn't make enough amount of blood cells.

Aplastic anemia develops when there is damage to bone marrow stem cells. When the cause remains unknown, it is known as idiopathic aplastic anemia.

Thus the causes of the disorder other than idiopathic fall into the "acquired" or "inherited" categories.

Acquired aplastic anemia (more common than the inherited form)

• Exposure to toxins like benzene an ingredient in gasoline, pesticides and arsenichave been linked to aplastic anemia.
• Radiation and chemotherapy treatments- when cancer-fighting therapies kill cancer cells, they can also damage healthy cells, including stem cells in bone marrow.
• Infectious diseases- viral infections play animportant role in the development of aplastic anemia in some people. including hepatitis, HIV and Epstein-Barr virus (a type of herpes virus)
• Auto immune disorder- lupus,rheumatoid arthritis, or other autoimmune disorders (those in which the immune system attacks healthy cells)
• Pregnancy
• Drugs-some antibiotics,immunosuppressants, and some NASIDs
• Cancer which had spread in bone

Inherited aplastic anemia is rare and develops from genes that are passed down to next generation, includefanconi anemia, dyskeratosiscongenital, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome

Clinical Sysmptoms And Signs

• Nausea
• Skin rashes
• Anemia (low red blood cell count), neutropenia (low white blood cell count), or thrombocytopenia (low platelet count)
• Fatigue
• Shortness of breath and chest pain
• Dizziness, especially after standing up from a sitting or lying position
• Pale skin
• Headaches
• Weakness
• Cold feeling in your hands and feet
• Increased heart rate.

Thrombocytopenia may cause:

• Bleeding that's difficult to stop
• Easy bruising and bleeding
• Tiny red dots on the skin
• Nosebleeds, bleeding gums, bloody stool, or heavy menstrual bleeding.

Diagnosis

In aplastic anemia, the patient has pancytopenia (i.e., less blood count of all blood cells) - leukopenia and thrombocytopenia.

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

Treatment

Treatment is usually only necessary in severe cases of aplastic anemia that are causing life-threatening symptoms.

• Blood Transfusion Platelet transfusions are the first course of treatment for any aplastic anemia patient who is severely deficient in platelet cells, thereby reducing the chance of fatal hemorrhage. Also, transfusions of red blood cells can help fighting the fatigue and shortness of breath. The procedure usually takes about one to two hours, depending on the blood component(s) you are receiving. Possible side effects include fever, allergic reaction or infection.
• Bone Marrow Transplantation The patient's non-functioning bone marrow is destroyed with drugs and/or radiation and replaced with donor bone marrow from a compatible donor, usually a sibling or other family member. If successful, bone marrow transplant can cure aplastic anemia with few relapses. However, serious immune complications and infections may happen including sterility and temporary hair loss.
• Medical Therapy Immunosuppressive drug therapy with anti-thymocyte globulin (ATG) and cyclosporine may be recommended for patients for whom bone marrow transplant is not an option. While drug therapy is not a cure for aplastic anemia, it may partially restore blood cell production. However, there may be 50% chance of relapse or developing other blood-forming malignancies or disorders. Side effects include fever, high blood pressure and kidney problems.
• Supportive care Supportive care and long term follow-up are imperative in the management of aplastic anemia. A central venous catheter – a tube that carries medicines to a vein in the chest – may be helpful for aplastic anemia patients who require frequent transfusions or infused medications.Because fungal and bacterial infections are quite common in patients with severe aplastic anemia, patients may require antibiotics, antiviral or antifungal agents.