Beta lifelong iron chelation therapy to prevent or reverse

Beta thalassaemia is a common inherited blood
disorder that results from genetic mutations with severe reduction or absent
production of the ?-globin chain of the hemoglobin tetramer. This results in
ineffective erythropoiesis caused by an excess of ?-globin chains and profound
anemia that is life-threatening from ?1 to 2 years of age1, 2 . Beta
thalassaemia is associated with severe hemolytic anemia that requires frequent lifelong
blood transfusions, often on a monthly or bi-monthly basis3, however, the iron load of ?200 mg per unit
combined with mildly increased gastrointestinal iron uptake related to hepcidin
suppression4 increases total body iron, which leads to a
requirement for lifelong iron chelation therapy to prevent or reverse
iron-related complications1, 5.


Iron overload leads to excessive iron deposition
in a wide variety of tissues, including the heart, the liver, and endocrine
tissues6–8, cardiac complications are the most
important, the major determinant of survival in patients with thalassaemia, and
responsible for more than half of the deaths in this population9. Therefore, the heart is the target lethal
organ in thalassemia. Once heart failure develops, the outlook is usually poor10–12.

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Although chelation therapy can reduce the
iron burden in patients with iron overload, toxicity and expense limits its
widespread use in developing and undeveloped countries, where the global burden
from iron overload is particularly high11, 13, 14. Therefore, several studies are frequently presented for the development
of new treatment strategies for increasing survival and improving quality of
life for iron-overload patients.


Previous in vitro studies showed that L-type
calcium channels provide a major pathway for iron entry into cardiomyocytes and,
in mice, calcium channel blockers reduce myocardial iron overload6,
11. Amlodipine is an inexpensive, widely
available calcium-channel blocker (CCB) with a well-known safety profile in
both adults and children15.


Spirulina is a multicellular filamentous
cyanobacterium (blue–green alga) with a long history of use as a
well-recognized food supplement for humans and animals16.
Spirulina is a rich source of proteins and vitamins, especially vitamin B12,
minerals, essential fatty acids, carotenoids and phycocyanins17. Although Spirulina is used in many
countries as a nutritional supplement, recently more attention has been paid to
its therapeutic potential. Spirulina have shown hypolipidemic18, hypoglycemic19, antihypertensive20,
21, anti-viral22, liver-protecting, anti-cancer,
anti-inflammatory and antioxidant properties23; as well as positive effects on immunity17, 24, 25.
Moreover, Spirulina is not expensive, has no side effects and is not toxic in


The cardiomyopathy may be
reversible if iron chelation treatment is intensified in time, but the
diagnosis is often delayed by the unpredictability of cardiac iron deposition
and the late development of symptoms and echocardiographic abnormalities26. However, cardiac MR (CMR) is considered the
gold standard investigation for early detection of cardiac iron overload and
can reliably identify patients with subclinical cardiac iron concentrations and
stratify their risk of subsequent cardiac dysfunction27,


The use of clinical magnetic resonance
imaging (MRI) to assess the extent of iron loading in organs has revolutionized
the diagnosis, management and treatment of beta thalassaemia patients29. MRI has two primary advantages over other
imaging or invasive diagnostic techniques; accurate measurement of cardiac
structure and function; and direct quantification of iron loading due to
shortening of magnetic relaxation times T1, T2 and T2* in the presence of iron.
These properties make it the ideal tool for clinical monitoring of beta thalassaemia
patients providing noninvasive monitoring of cardiac iron deposition and the
consequential iron induced cardiomyopathy30–32.

In the present study, we investigated the use of the calcium
channel blocker Amlodipine and Spirulina as novel and complementary treatment
to standard chelation therapy in reducing iron overload in patients with TM
using MRI. 


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