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Iron and Sickle Cell Disease

Iron and Sickle Cell Disease

Iron deficiency in a sickle cell patient is often accompanied by an absence of bone marrow from low serum ferrite level and hypochromic, microcytic ribonucleic cells. Furthermore, it also leads to decreased mean corpuscular haemoglobin concentration and an intense scarcity of sickled erythrocytes in blood smears. Iron deficiency in sickle cell disease patients is primarily caused by excess urinary loss of iron secondary to chronic intravascular hemolysis.

As much as there are consequences of iron deficiency in the body of sickle cell patients, iron overload can also lead to severe consequences, which reflects in the distribution of iron storage. The main source of iron overload is blood transfusion. In fact, in contrast to other inherited cases of sickle cell disease, iron overload rarely occurs without blood transfusion. In a reduced blood transfusion rate, storage iron hardly ever exceeds 2000mg in sickle cell disease present majorly in macrophages of the liver, the spleen and BM.

In a survey conducted in India, it was found that iron deficiency was common in women with sickle cell disease than those with the traitor had healthy controls. This further buttress the fact that iron overload occurs when there is repeated blood transfusions.

Transfusional iron overload is a significant clinical issue in sickle cell disease. Iron overload was present in approximately one-third of 141 adult SCD patients at post mortem (mean age, 36 y), and 7% of deaths were judged to be related to iron overload. Haematology, ASH Education Program 2013, Consequences and management of iron overload in sickle cell disease,

LIC, fibrosis and cirrhosis are some of the consequences of iron overload in sickle cell patients. Portal fibrosis can set in if transfusion is given without chelation.

 


Monitoring Iron Overload in Sickle Cell Disease

Serum Ferritin (SF) is one of the widely used tests to determine the rate of iron overload, though it has some limitations. LIC and MRI techniques are also perfect and effective methods/tools for monitoring iron overload. Plasma markers of iron overload, Fibroscan with blood markers etc., also offer close monitoring of iron overload.

Instead of carrying out transfusion, the following treatments can be carried out on the SCD patients:

·         Non-Pharmacological Therapy: This is one method of tackling iron overload as erthracytapharesis can be substituted with simple transfusions of younger red cells and carrying out splenectomy in hypersplenism patients. When young erythrocytes known as neophytes are transfused, there is a significant decrease in transfusion requirements and often leads to prolonged transfusion interval by 15-25%. Though this tends to be more expensive, and the donor is more exposed.

·         Pharmacological Therapy: This is the use of chelators to target the unbound iron in the blood that cause tissue injury.

In conclusion, iron overload a feared complication in sickle cell disease, and as transfusions in SCD continues to skyrocket, it is pertinent to state here that unnecessary transfusions in patients suffering from SCD should be strictly discouraged or minimised to decrease the complications of iron overload.

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