Introduction:
Iron is an essential micronutrient, the role of iron in the human body is beyond compare. Iron is vital for development, and it is a significant content of hemoglobin, a protein present in the red blood cells. The human body is all about perfection, and it can process every nutrient if present in the correct quantity. Too much or too little of something can disturb this balance and lead to adverse outcomes. It is common to encounter diseases like anemia due to iron deficiency, but one would never imagine that too much iron is bad for the human body. Yes, the human body can not handle too much iron. This condition is known as hemochromatosis or iron overload.
What Is Secondary Hemochromatosis?
The total body iron pool ranges from two to six grams in healthy adults. 98 % of this is stored in cells of the liver. Secondary hemochromatosis is characterized by excessive accumulation of iron in the body. Most of this excessive iron gets deposited in organs like the liver and pancreas. Unfortunately, humans do not have a major pathway for iron excretion. As a result, excessive iron in my body gets absorbed into the system. Hemochromatosis can be acquired or hereditary. Acquired forms of hemochromatosis with known sources of excess iron is known as secondary hemochromatosis.
What Causes Secondary Hemochromatosis?
The main cause of secondary iron overload can be attributed to excessive iron intake in the diet or blood transfusions. Iron overload because of excessive intake in the diet is relatively rare. The reasons can be broadly divided into the following categories.
A. Parenteral Iron Overload Due to Transfusions - This is a secondary complication associated with the treatment of anemias. When the oral iron supplements are insufficient to bring the iron levels to a normal range, parenteral iron preparations are given. In worst-case scenarios, the patient might have to undergo a blood transfusion to make up for the deficiency.
Some of the disorders needing a blood transfusion or parenteral iron therapy include:
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Aplastic anemia.
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Sickle cell anemia.
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Leukemias.
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Sideroblastic anemia.
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Severe iron deficiency anemia.
B. Chronic Liver Disease - Alcoholic liver cirrhosis (end-stage scarring or fibrosis of the liver due to excessive alcohol intake) is frequently associated with iron deposition in the liver cells. In this condition, the total body iron is not increased. Instead, it represents an alcohol-induced redistribution of iron. Long-standing liver disease affects iron metabolism, which results in iron overload or secondary hemochromatosis.
C. Increased Iron Intake Through Diet: Bantu Siderosis - This rare condition is seen in sub-Saharan Africa. Interestingly, iron overload occurs as a result of ingesting large quantities of alcoholic beverages fermented in iron utensils. It is a genetic susceptibility that has been identified in this population due to the home brewing of alcoholic beverages in steel drums.
D. Long-Term Hemodialysis - It is a rare complication seen in patients with end-stage kidney disease. Due to multiple rounds of dialysis, the patient develops anemia. To manage anemia, drugs that stimulate the production of red blood cells are given. Along with this, iron supplements are given in the form of injections. A very low frequency of patients develop iron toxicity due to excessive iron supplementation.
What Is the Difference Between Primary And Secondary Hemochromatosis?
Primary hemochromatosis is hereditary. It affects males more than females and rarely becomes evident before the age of 40. In hereditary hemochromatosis, the iron accumulation can exceed 50 gm. Hereditary hemochromatosis is a homozygous recessive (if it carries two copies of the same recessive allele) inherited disorder. On the other hand, secondary hemochromatosis is an acquired condition. It is almost always linked to known sources of excess iron intake.
What Are the Risk Factors for Developing Secondary Hemochromatosis?
Some people are more likely to develop this condition. Old age, alcoholism, and a family history of heart attacks, liver disease, diabetes are some of the known risk factors.
What Are the Symptoms of Secondary Hemochromatosis?
Secondary iron overload can exhibit a plethora of symptoms. The symptoms can range from simple weakness to heart failure in extreme cases. It is important to note that not everyone with secondary iron overload develops symptoms. Patients can be asymptomatic for several years before developing symptoms. The symptoms usually appear post 40 years of age. The symptoms can be broadly classified into the following categories.
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General Symptoms - This includes weakness, tiredness, and unexplained weight loss.
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Musculoskeletal - Joint pain, especially in the knees and hands. Symptoms resemble atypical arthritis. Pain in the knuckles of fingers, known as the iron fist.
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Skin - Grey, metallic, or bronze pigmentation of the skin can be seen particularly in sun-exposed areas.
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Endocrine - Diabetes or glucose intolerance can be seen in some patients. Hypothyroidism is another common finding.
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Gastrointestinal - Abdominal pain is a prevalent complaint. Liver disease can be a fatal complication in long-standing cases.
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Gonads - Some patients present with the complaint of hypogonadism (failure of hormone production in testes and ovaries). Some patients also complain of erectile dysfunction caused due to iron deposition in gonads. Amenorrhea or absence of menses in females and loss of libido in males is common.
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Cardiovascular - In extreme cases, patients experience irregular heartbeats and cardiomyopathy. Chances of heart failure are possible but rare.
How Is Secondary Hemochromatosis Diagnosed?
Patients may require a blood test, liver biopsy, and MRI (magnetic resonance imaging) to establish the diagnosis. Serum ferritin, iron, transferrin saturation, total iron-binding capacity is evaluated. Further diagnosis depends on the presenting symptoms, physical examination, and other relevant tests.
What Is the Treatment for Secondary Hemochromatosis?
The first step in treating secondary iron overload is identifying the cause. As mentioned earlier, multiple factors can lead to iron toxicity.
A. Modifying the Diet - Avoiding foods rich in iron content, and reducing alcohol intake can help upto some extent. Vitamin C supplements should be avoided.
B. Iron Chelation Therapy - To remove the extra iron from the body, chelating agents (substances that can bond with metal ions) can be given orally or intravenously. Some examples of iron-chelating agents include Deferoxamine, Deferiprone, Deferasirox.
C. Phlebotomy - It is the process of drawing blood from the veins using a cannula. Therapeutic phlebotomy is used as an adjunct treatment. Patients may require multiple rounds of therapeutic phlebotomy in order to bring the iron levels down.
What Is the Outcome or Prognosis of the Treatment?
Secondary hemochromatosis has a favorable prognosis if detected early. If the iron levels are effectively reduced, the symptoms subside. If the condition is left untreated, the outcomes can be fatal. If there is damage to any organs, further treatment can be modified to reduce the morbidity.
Conclusion:
Secondary hemochromatosis is not a fatal condition. But having said that, early detection and treatment of this condition are crucial. If detected early, the disease can be managed easily. If not, the disease can progress and lead to complications such as liver cirrhosis and heart failure. If no complications develop, the patients show longevity and excellent recovery.
