Transfusional hemosiderosis is the accumulation of iron in the body due to frequent blood transfusions. Iron accumulates in the liver and heart, but also endocrine organs. Frequent blood transfusions may be given to many patients, such as those with thalassemia, sickle cell disease, leukemia, aplastic anemia, or myelodysplastic syndrome, among others. It is diagnosed with a blood transferrin test and a liver biopsy. It is treated with venipuncture, erythrocytapheresis, and iron chelation therapy.
| Transfusion hemosiderosis | |
|---|---|
| Specialty | Hematology |
| Causes | Frequent blood transfusion |
| Diagnostic method | Blood transferrin test, liver biopsy |
| Differential diagnosis | Other causes of hemosiderosis |
| Treatment | Phlebotomy, erythrocytapheresis |
| Medication | Iron chelation therapy |
Signs and symptoms
editTransfusional hemosiderosis can cause cardiac arrhythmia and cardiomyopathy.[1]
Causes
editTransfusional hemosiderosis is a potential side effect of frequent blood transfusions.[2] These may be given for a number of conditions, including:
Mechanism
editHemoglobin, the oxygen-carrying molecule in a red blood cell, contains iron. The body has limited ways to store and remove iron. When red blood cells (RBCs) die, they are consumed by macrophages. 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 the liver, heart, pancreas, and endocrine organs.[2] Free iron increases the production of oxygen radicals (mostly hydroxyl radicals) that cause damage to cells (particularly their DNA).[2]
Diagnosis
editTransfusional hemosiderosis can be inferred with a blood transferrin test. Blood ferritin may be increased with a number of other conditions, so is less reliable for diagnosis.[4] A liver 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]
Treatment
editTransfusional hemosiderosis is treated with a number of therapies. Venipuncture (phlebotomy) removes blood. Erythrocytapheresis filters red blood cells from the blood. Chelation therapy removes iron from the blood.[5] This involves delivering iron chelating agents such as deferoxamine, deferiprone or deferasirox.[5] If iron overload has caused damage to end-organs, this is generally irreversible and may require transplantation.[clarification needed]
Prognosis
editTransfusion 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 and cardiac arrhythmia are often a cause of death.[1]
Society
editTed DeVita died of transfusional iron overload from too many blood transfusions.[citation needed]
See also
editReferences
edit- ^ a b Hernando, Diego; Levin, Yakir S.; Sirlin, Claude B.; Reeder, Scott B. (2014). "Quantification of liver iron with MRI: State of the art and remaining challenges". Journal of Magnetic Resonance Imaging. 40 (5): 1003–1021. doi:10.1002/jmri.24584. ISSN 1522-2586. PMC 4308740. PMID 24585403.
- ^ a b c d e f g h i Shander, A.; Cappellini, M. D.; Goodnough, L. T. (2009). "Iron overload and toxicity: the hidden risk of multiple blood transfusions". Vox Sanguinis. 97 (3): 185–197. doi:10.1111/j.1423-0410.2009.01207.x. ISSN 1423-0410. PMID 19663936.
- ^ Sharma, Deva; Ogbenna, Ann Abiola; Kassim, Adetola; Andrews, Jennifer (April 2020). "Transfusion support in patients with sickle cell disease". Seminars in Hematology. Transfusion Support in Patients with Hematologic Disease: Transfusions in Special Clinical Circumstances. 57 (2): 39–50. doi:10.1053/j.seminhematol.2020.07.007. ISSN 0037-1963. PMID 32892842. S2CID 221523056.
- ^ a b Borgna-Pignatti, C; Castriota-Scanderbeg, A (September 1991). "Methods for evaluating iron stores and efficacy of chelation in transfusional hemosiderosis". Haematologica. 76 (5): 409–413. ISSN 1592-8721. PMID 1806447.
- ^ a b Hider, Robert C.; Kong, Xiaole (2013). "Chapter 8. Iron: Effect of Overload and Deficiency". In Astrid Sigel, Helmut Sigel and Roland K. O. Sigel (ed.). Interrelations between Essential Metal Ions and Human Diseases. Metal Ions in Life Sciences. Vol. 13. Springer. pp. 229–294. doi:10.1007/978-94-007-7500-8_8. ISBN 978-94-007-7499-5. PMID 24470094.