Beta Talasemi Major Hastalarinda Oral Deferasirox ve Diğer Demir Şelasyon Tedavilerinin Kardiak, Hepatik ve Endokrin Organlardaki Demir Birikimi Üzerine Etkisinin T2* Mrg ile Değerlendirilmesi

Abstract

Thirtyseven patients with β thalassemia major who were being followed up in Hacettepe University, İhsan Doğramacı Children’s Hospital, Division of Pediatric Hematology and on the same study drugs for at least 1 year were included. Cardiac, hepatic T2*, pancreas T2* and R2*, pituitary, thyroid T2 and R2 MRI were oredered twice with a median interval of 6 months (6-11 months. The patients were evaluated in terms of complete blood count, serum biochemistry and endocrinological tests, as well. The patients were grouped into two according to iron chelation type: deferasirox group and other chelators group. The groups were compared according to age, gender, splenectomy status, body weight percentile, height percentile, body mass index, laboratory parameters, and MRI T2* or T2 and/or R2* or R2 values, and the changes (δ-delta) in these parameters were evaluated. Of the study group, 54.9% of the patients were found to have osteopenia, 8.1% had hypothyroidism, 8.1% had hypogonadotropic hypogonadism and 7.1% had diabetes mellitus. No statistically significant difference was identified between drug groups in terms of hypogonadism, hypothyroidism, hypopituitarism and osteoporosis. Almost all of the patients in the study group were found to have pituitary iron deposition according to the previously determined cut-off values with measurements from the healthy controls. In both drug groups, the iron accumulation in thyroid gland was found to increase in the second MRI measurements. Despite the limiting factors of small sample size and short follow-up time, deferasirox and the other drug group were found to have similar iron chelation effect. Among the all patients included in the study, pancreas R2* and cardiac T2* values were found to have a negative correlation, indicating increased iron accumulation in pancreas in parallel with cardiac iron accumulation. Fasting blood glucose level and T2* pancreas MRI measurements were found to be in negative correlation, significantly. On the vii other hand, fasting insulin and R2* pancreas MRI were found to be in positive correlation. This indicated that the increase in pancreas iron overload was in paralel with the increase in fasting insulin. A negative correlation was found between hepatic T2* value and HOMA-IR, indicating the relationship between increased hepatic iron and insulin resistance. Our results indicated that endocrinopathy rates are common among the patients with β thalassemia major and the importance of follow-up of these patients with blood tests and T2* or T2 and/or R2* or R2 for the detection of iron status in the endocrine organs, in addition to cardiac and hepatic T2* evaluations. Since cardiac T2* measurements are more commonly evaluated in these patients, the patients who were found to have cardiac iron loading in these evaluations should be more meticulously tested for insulin resistance and diabetes mellitus. Furthermore, the patients who were found to have hepatic iron loading were suggested to be tested for insulin resistance, especially with HOMA-IR measurements.