Frontiers in Clinical Drug Research: Hematology

Author(s): Tirin Babu and George Mathew Panachiyil * .

DOI: 10.2174/9789815039535122050004

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An Evidence-Based Approach to Treatment with Iron Chelators in Transfusion- Dependent Thalassemia Patients : Present Trends and Future Scenario

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Abstract

SHS investigation development is considered from the geographical and historical viewpoint. 3 stages are described. Within Stage 1 the work was carried out in the Department of the Institute of Chemical Physics in Chernogolovka where the scientific discovery had been made. At Stage 2 the interest to SHS arose in different cities and towns of the former USSR. Within Stage 3 SHS entered the international scene. Now SHS processes and products are being studied in more than 50 countries.

Abstract

Treatment of hemochromatosis is a significant target-based care of
transfusion-dependent thalassemia and non-transfusion dependent thalassemia patients.
In some cases hemochromatosis is a secondary concern to frequent red blood cell
transfusions as in transfusion-dependent thalassemia (TDT) or advances from enhanced
gastrointestinal iron absorption such as in non-transfusion dependent thalassemia
(NTDT), this can cause serious illness and death to the patients. When thalassemia
major patients undergo frequent blood transfusions, hemochromatosis is unavoidable
because the human body lacks a physiological mechanism to evacuate extra iron.
Thalassemia patients with transfusional hemochromatosis regularly need treatment
with iron chelators to decline the iron overload and thereby retard long-term effects
related to iron accumulation in tissues. Deferoxamine, deferiprone, and deferasirox are
the three currently approved iron chelators for the treatment of hemochromatosis in
transfusion-dependent thalassemia patients. Today, iron chelation therapy's goal is to
sustain acceptable levels of iron in the human body at all times. Correct tailoring with
iron chelators and their dose modifications must implement on time.
We first describe the pathophysiology of hemochromatosis in thalassemia patients in
this chapter. We then cover iron chelation therapy's general goals, the features of the
permitted iron chelators, and the evidence-based practice behind the usage of iron
chelators as a single drug, or as part of combination therapy, and the mechanisms by
which chelators work. The guiding principles for monitoring treatment with iron
chelators to reduce the toxicity risks from iron chelation are later explained. Finally, the
importance of deferasirox twice-daily dose instead of a once-daily dose in transfusiondependent
thalassemia patients with inadequate response to high doses and the future
directions in treating iron overload in thalassemia patients is discussed.

Keywords:

Alpha Thalassemia, Beta-Thalassemia, Blood Transfusions, Combination Therapy, Deferasirox (DFX), Deferiprone (DFP), Deferoxamine (DFO), Exjade, Intolerance, Iron Chelation Therapy, Iron Chelator, Iron Overload, Jadenu, JAK2 Inhibitors, Luspatercept, Minihepcidins, Monotherapy, Non-Transfusion Dependent Thalassemia (NTDT) Patients, Novel Oral Iron Chelator, Poor Response, Serum Ferritin, Sotatercept, Thalassemia Guidelines, Transfusion Dependent Thalassemia (TDT) Patients, Twice-Daily Deferasirox.

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