What Is Sideroblastic Anemia?
Hemoglobin is a pigment present in the blood that plays an assistive role in delivering oxygen to every part of the body. When the iron present in the red blood cells or erythrocytes is not productively used to synthesize hemoglobin, it results in sideroblastic anemia. Consequently, hemoglobin production is significantly affected, leading to an inadequate supply of oxygen to vital organs like the heart, lungs, liver, brain, etc. It thereby produces serious health problems. As a result of inadequate usage, the excess iron gets built up in the red blood cells around the nucleus in the form of a ring or sideroblast. Thus, the name ‘ sideroblastic anemia.’
How Is Sideroblastic Anemia Classified?
Based on its origin, sideroblastic anemia is divided into three types, namely.
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Genetic Sideroblastic Anemia - It is caused due to genetic mutation.
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Acquired Sideroblastic Anemia - This form of the disease occurs due to toxin exposure, nutritional deficiencies, or other chronic health problems and usually shows symptoms above the age of 65 years.
When the cause of sideroblastic anemia cannot be determined, it is termed idiopathic.
What Is Acquired Idiopathic Sideroblastic Anemia?
Acquired idiopathic sideroblastic anemia is a rare myelodysplastic syndrome that occurs due to ineffective heme synthesis and hematopoiesis (blood cell production) which in turn, results in cytopenias (reduced level of blood cells) and leukemia (cancer that occurs in the blood-forming tissues).
How Prevalent Is Acquired Idiopathic Sideroblastic Anemia?
It has no gender preference and equally affects both men and women. The rate of incidence for a year in the European nations is around one in every one lakh individuals. Older adults above the age of 65 years are most commonly affected.
What Is the Cause of Acquired Idiopathic Sideroblastic Anemia?
The exact cause of the disease is not clearly known. It has been attributed to be a clonal disorder that occurs due to an abnormality in the hematopoietic stem cell. In acquired sideroblastic anemia, the genetic mutation does not usually happen. However, a few cases have reported chromosomal derangement in the Xq13 gene.
In addition to the role of genetic mutations in affecting the treatment of acquired idiopathic sideroblastic anemia, lifestyle factors and diet also can have an impact.
What Are the Symptoms of Acquired Sideroblastic Anemia?
The following are the symptoms of acquired idiopathic sideroblastic anemia:
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Fatigue.
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Pale skin.
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Enlargement of spleen or liver.
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Palpitations or increased heart rate.
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Shortness of breath.
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Fussiness.
How Is Acquired Idiopathic Sideroblastic Anemia Diagnosed?
Diagnosis is made initially by assessing the medical history and family history of the patient.
It is then followed by blood examination and bone marrow examination.
- Blood Examination: A complete blood count is done, which detects the level of hemoglobin, red blood cells, white blood cells, and other essential blood markers. When there is an abnormality, a peripheral blood smear is suggested. A peripheral blood smear helps identify the sideroblastic appearance of the red blood cells.
- Bone Marrow Examination: In bone marrow examination, a piece of tissue is removed and is checked for the presence of any cancer or blood disorders. In the case of acquired idiopathic sideroblastic anemia, more than 15% are ringed sideroblasts of the total amount of erythrocytes. Bone marrow aspiration is also done to diagnose acquired idiopathic sideroblastic anemia, in which a needle is inserted into the bone to remove a small amount of bone marrow.
- In acquired idiopathic sideroblastic anemia, there is no dysplasia (abnormal cell count) seen in other blood cell types. The number of myeloid blasts (immature myeloid cells or subgroups of WBC) is less than 5%.
What Is the Treatment for Acquired Idiopathic Sideroblastic Anemia?
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Supportive treatment is suggested for acquired idiopathic sideroblastic anemia to reduce the symptoms associated with it.
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In conditions where there is an increased level of iron in the blood, a reduction in the iron level can help return the blood cells to their normal form and function. Regardless of any other treatment, reducing iron levels is essential. Oral iron chelating agents like Deferiprone and Deferasirox help eliminate the excess iron present in the body.
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If any drug is identified as the cause of acquired idiopathic sideroblastic anemia, replacing that drug with any other alternative is needed. However, it should be done only at the advice of the treating doctor.
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Vitamin B-6 or Pyridoxine therapy may be helpful in the treatment of acquired idiopathic sideroblastic anemia. At times, when it becomes less effective, red blood cell transfusion may be recommended.
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There may be a need for bone marrow transplantation or stem cell transplantation in severe cases of acquired idiopathic sideroblastic anemia.
What Is the Role of Diet in Acquired Idiopathic Sideroblastic Anemia?
Biochemical pathways are affected by the foods taken. Certain foods enhance the treatment by offering favorable results, while some can have adverse interactions or increase the progression of the disease. The dietary modifications are dependent on the type of treatment, age, gender, and intake of supplements.
What Is the Prognosis of Acquired Idiopathic Sideroblastic Anemia?
In the case of acquired idiopathic sideroblastic anemia, there is only less than a 5 % chance of the disease progressing to leukemia. Also, the rate of prognosis is reasonable even in individuals above the age of 70 years. There is not much difference in the prognosis rate in older adults compared to the general population. The etiological factors and underlying health conditions play a decisive role in determining the prognosis.
Conclusion:
Acquired idiopathic sideroblastic anemia is a rare myelodysplastic disorder. However, with appropriate treatment methods, it can have a good prognosis. Lifestyle modifications, dietary modifications, and proper treatment methods can give the individual a prolonged lifespan.
