| Transfusion hemosiderosis | |
|---|---|
| Specialty | Hematology |
| Causes | Frequentblood transfusion |
| Diagnostic method | Bloodtransferrin test,liver biopsy |
| Differential diagnosis | Other causes ofhemosiderosis |
| Treatment | Phlebotomy,erythrocytapheresis |
| Medication | Ironchelation therapy |
Transfusional hemosiderosis is the accumulation ofiron in the body due to frequentblood transfusions. Iron accumulates in theliver andheart, but alsoendocrine organs. Frequent blood transfusions may be given to many patients, such as those withthalassemia,sickle cell disease,leukemia,aplastic anemia, ormyelodysplastic syndrome, among others. It is diagnosed with a bloodtransferrin test and aliver biopsy. It is treated withvenipuncture,erythrocytapheresis, and ironchelation therapy.
Transfusional hemosiderosis can causecardiac arrhythmia andcardiomyopathy.[1]
Transfusional hemosiderosis is a potential side effect of frequentblood transfusions.[2] These may be given for a number of conditions, including:
Hemoglobin, the oxygen-carrying molecule in ared blood cell, contains iron. The body has limited ways to store and remove iron. When red blood cells (RBCs) die, they are consumed bymacrophages. Transfused RBCs have shorter lifespans that native ones, so they die and are consumed more frequently by the macrophages, which causes the latter to die from excess iron which is then released into the blood.[2] Therefore, with frequent blood transfusions, iron builds up in the body over time.[2] This can enter theliver,heart,pancreas, andendocrine organs.[2] Free iron increases the production ofoxygen radicals (mostlyhydroxyl radicals) that cause damage to cells (particularly theirDNA).[2]
Transfusional hemosiderosis can be inferred with a bloodtransferrin test. Bloodferritin may be increased with a number of other conditions, so is less reliable for diagnosis.[4] Aliver biopsy may be used, which is the most accurate diagnostic technique.[4] The level of siderosis seen in a liver biopsy can be graded by severity.[2]
Transfusional hemosiderosis is treated with a number of therapies.Venipuncture (phlebotomy) removesblood.Erythrocytapheresis filtersred blood cells from the blood.Chelation therapy removes iron from the blood.[5] This involves delivering ironchelating agents such asdeferoxamine,deferiprone ordeferasirox.[5] If iron overload has caused damage to end-organs, this is generally irreversible and may requiretransplantation.[clarification needed]
Transfusion hemosiderosis can cause permanent damage to tissues that may lead to death.[2] Tissue damage can remain even after chelation therapy.[2] Outcomes are usually worse in patients who require blood transfusions compared to those who can have alternative therapies.[2]Cardiomyopathy andcardiac arrhythmia are often a cause of death.[1]
Ted DeVita died of transfusional iron overload from too manyblood transfusions.[citation needed]