Afshan Shirkavand; Zahra Razaghi; Sharam Akhlaghpoor; Azita Azarkeivan; Mehran Karimi
Abstract
Introduction: Multi-organ iron load is prevalent crucial side effect in thalassemic patients due to repeated transfusions, and high intestinal iron absorption. MRI T2* has demonstrated its potency as a non-invasive technique for the imaging of hemosiderosis in thalassemia. We aim to investigate the iron ...
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Introduction: Multi-organ iron load is prevalent crucial side effect in thalassemic patients due to repeated transfusions, and high intestinal iron absorption. MRI T2* has demonstrated its potency as a non-invasive technique for the imaging of hemosiderosis in thalassemia. We aim to investigate the iron load of adrenal glands and kidneys using MRI T2* in adult thalassemia patients and evaluate the serum ferritin correlation of with kidneys, heart, liver, and adrenal glands’ iron load.Methods: Thirty-five thalassemia major (TM) and thalassemia intermediate (TI) patients (age range 18-50 years) from Zafar thalassemia Clinic, were recruited in this survey from September 2019 to October 2020. Magnetic Resonance Imaging (MRI) was used to map iron overload in several organs’ regions of interest (ROIs) using fast-gradient-echo multi echo T2*sequences protocol. T-test and chi-square analysis were done.Results: Nine (25.7%) patients had left Kidney T2* less than 36ms which could indicate abnormal renal iron load while this was 8 (22.9%) for the right kidney. In the left and right adrenal glands, these numbers were 31 (88.6%) and 29 (82.9%), respectively, below the normal threshold.Conclusion: Adrenal gland and renal iron overloads were detected in MRI images of thalassemic patients. Correlation for serum ferritin levels and kidney and adrenal glands T2* was found weakly negative. Non-invasive monitoring of the internal organs’ hemosiderosis using MRI T2* was found to be beneficial for iron-chelating optimization and preventing irreversible tissue damage.
Afshan Shirkavand; Leila Ataie Fashtami; Azita Azarkeivan; Zohreh Zahedi
Abstract
Introduction: Thalassemia consists of a variety of genetic hemoglobinopathies. Thalassemia-major causes anemia in early age. Those suffering from thalassemia need frequent life-long blood transfusions to survive, resulting in iron overload in the body and many health problems. Much improvement has occurred ...
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Introduction: Thalassemia consists of a variety of genetic hemoglobinopathies. Thalassemia-major causes anemia in early age. Those suffering from thalassemia need frequent life-long blood transfusions to survive, resulting in iron overload in the body and many health problems. Much improvement has occurred in predicting the course of Thalassemia major thanks to iron chelation therapy. Edible iron chelating agents are the standard of the chelating process. Deferasirox is a newly developed orally active iron chelating tablet which is used on a daily basis. Th present case study investigated severe dermatopathological reactions to the Iranian made product of Deferasirox.Case presentation: We present a case of adverse drug reactions in a thalassemic patient who was started on Deferasirox orally after receiving Deferoxamine injections for several years with no serious reactions. The patient experiences generalized maculopapular, deep red- blue partially purpuric itchy skin rashes throughout her body. The histopathological biopsy found superficial perivascular or dermatitis with low-grade vasculopathy, few eosinophils, and mild psoriasis form-supraglotticlichenoid epidermal reactions associated with Drug Reaction diagnosis.Conclusion: With regard to inherent features, caution must be applied to start the original Deferasirox for the patients who will undergo the oral chelation process with a smooth increase in the daily dosage for a few weeks in order to create improved tolerance.