Sideroblastic Anemia – Prognosis
In treating sideroblastic anemia, the first step involves the ruling out of reversible causes such as drug or substance abuse, alcoholism, and exposure to toxins like lead and zinc. Blood transfusion is one of the main procedures being done when trating this disorder. This is done to ensure that the body of the patient will maintain a stable amount or level of hemoglobin. Treatment with pyridoxine is optional, and can be used in rare cases where it is most beneficial to the patients.
For patients who are dependent on blood transfusions, and with additional conditions in which pyridoxine and other forms of therapy is not beneficial to their case, the possibilities of being cured are quite dim. Especially for those who have developed acute myelogenous leukemia, treatment with vitamin B complex may prove to be ineffective.
For sideroblastic anemia which is congenital, the patient is also at risk for neuromuscular dysfunction. This is caused by the abnormalities in the mitochondria of the patient. For patients who have acquired sideroblastic anemia in their lifespan, the rate for mortality and morbidity may vary, especially since the causes for this anemia are reversible. For cases of idiopathic sideroblastic anemia, the average period for survival is 38months, while pure sideroblastic anemia has a median survival rate of 60 months.
Death rates and cases in sideroblastic anemia are attirubuted to leukemia and blood transfusions that cause hemochromatosis. Patients who die from this disease have developed acute leukemia in the later stages of the sideroblastic anemia. Their reticulocyte count is significantly lower, and their needs for blood transfusion are greater together with thrombocytopenia.
Sideroblastic anemia has only hundreds of cases around the world, most of which, greatly affects Americans. In Atlanta, Georgia, 13% of sideroblastic anemia has been found to occur in children aged 13 and below, and these numbers are from a hereditary type.
To maintain the hemoglobin levels in the body, a patient is required to undergo chronic transfusion on a regular basis. The basis of the transfusion should be the symptoms that are suffered by the patient, and not the absolute level of hemoglobin. If the latter is followed, the consequences of the transfusion may be adverse, and could further harm the patient instead of control the situation and treat them.
Monthly or yearly monitoring of the hemoglobin levels is also required, especially with patients who have inherited sideroblastic anemia directly from immediate relatives. As extensive research and development for the cure continues, these tests and monitoring procedures can greatly contribute to the treatment and prevention of this rare iron disorder. As the stages of sideroblastic anemiamay vary, so can the pathophysiology and etiology. The biochemical abnormalities are the common traits that this and other types of blood diseases share. As recent discoveries continue, our understanding for this disease is also increased.
In hereditary cases, 80% of patients with sideroblastic anemia were responsive to the treatment of vitamin B6, and have significantly increased their hemoglobin levels. In acquired cases, 40 percent of patients were responsive to the treatment, but their hemoglobin levels were not completely restored to normal.