Cardiac & Hepatic Iron overload are prevalent and understandable in transfusion-dependent SCD patients. however, it has been observed and reported by many recent cases about iron overload among non-transfusion dependent SCD patients; is there any studies or hypotheses about the justification of this overload?
Increased absorption of dietary iron induced by ineffective erythropoiesis. Similar mechanism also operates in other disorders of ineffective erythropoiesis such as thalassaemias, congenital dyserythropoietic anaemias (CDA) and myelodysplastic syndromes. They may respond to iron chelation like those on regular transfusion.
thank you Dr. Muttuswamy Sivakumaran for the valuable contribution
I would say ineffective erythropoiesis is not a feature of sickle cell anaemia unless a co-existing thalassaemia is inherited. The erythropoiesis in SCA is, reasonably, effective but the lifespan of the red cells compromised out side the bone marrow (intravascular or extravascular & splenic sequestration). Regarding the presence of iron overload in SCA, again I didn't came across such cases in pure SCA. Would you mind Abdulqadir to provide more detail where this has been reported? Regards.
I do agree with Mazin Al-Asadi ,, why there will be iron overload in cases with sickle cell anemia, without transfusion
Please find the following recent published article in Blood journal
Article Increased Liver Iron Concentration (LIC) in Adults with Non-...
I need the data whether the presented patients, with Non-Transfusion Dependent Sickle Cell Disease (NTD-SCD) and Increased Liver Iron Concentration (LIC), had isolated hepatic iron overload or had generalized siderosis (which should be confirmed with submucosal salivary gland biopsy).
Please, provide me with this information!
Sincerely,
Petar Ivanovski
The phenotype of the studied patient were not clear in this article. Were they HbSS or HBS-thalassemia? As both can be referred as Sickle cell disease (SCD) and both could have transfusion-free life. Those who carry the abnormal thalassemia gene are commonly associated with in-effective erythropoiesis and chronic stimulation of iron absorption from the GIT that might predispose to iron overload. Secondly it is important do declare that the studied patients had no history of chronic iron supplementation which is a treatment might, erroneously, be prescribed to any patient with hypochromic anaemia on the assumption that it is mostly due to iron deficiency. Regards.
thank you Peter and Mazin for the valuable questions .. i have invited Dr. Yassin the PI to give his respected opinion
RBC's have a standard life of 120 days. Stored blood will have a life of 50 days. Sickle cell crisis will cause cells to deform and block capillaries. HbS cells will have a tendency to lyse.and release Fe, and K. Also, of interest, LDH increases in these patients. One would think that this would be due to LDH 1 and 2 isomers. Interestingly enough, LDH 5 is elevated indicating an ischemic event more prominent than hemolytic event. Also, SCD patients will require transfusions. Transfused cells have a 50 day lifespan and therefore will release more iron. Washing the cells helps to some extent but not much. Attempts have been made to use "neocytes", namely younger cells with heavier specific gravity. This is a time consuming and expensive process. It takes about 5 units of whole blood to come up with one unit of neocytes. This practice has been abandoned many years ago. The iron released by the RBC's will deposit in the liver, heart and pancreas causing serious deterioration of function in these organs. Chelation therapy with Desferoxamine, and more recently Ex-Jade, are the treatment o fchoice. This is not something without its own risks... I hope this helps.
from a pratical point of vue, yes we've to actively look for iron surcharge in HbSS people : many have aditional alpha talassemia deletions, causing microcytosis - micro and anemia byitself may increase the iron absortion; even if they aren't transfusion dependent, they are transfusioned sometimes; at least in western countries diets trend to have high iron content; some still have poor muscle mass (and muscle can "harbour" a lot of iron); some of them will have heterozigoty for haemochromatosis; and the role of free iron from intravascular hemolysys as described
all that together make iron surcharge a serious issue, not so much for pediatrics, but for the adult group, which longevity is improving
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