Effects of Splenectomy on Natural Killer Cell Levels in ß-Thalassemia Major Patients.

AIM: In this study, we investigated how splenectomy affects natural killer (NK) cell levels in patients with ß-thalassemia major (ß-TM). MATERIALS AND METHODS: Seventy patients with ß-TM (38 splenectomized and 32 nonsplenectomized) and 25 healthy controls were included in this study. The hemogram parameters, ferritin, T lymphocyte, T-helper cell, T-suppressor cell, and NK cell numbers, were measured. RESULTS: The T lymphocyte (CD3+) level was found to be significantly higher in the patient group (p < 0.05). CD3+/CD4+ T lymphocytes were detected to be significantly higher in the patient group (p < 0.05). Although the CD3+/CD4+ T lymphocyte level was significantly higher in the nonsplenectomy group (p < 0.05), this was not the case in the splenectomy group. When the patient and control groups were compared, no significant difference was detected regarding CD3+/CD8+ T lymphocyte levels. CD3-/CD16+CD56+ NK cell level was found to be significantly lower only in the splenectomy group than in the control group (p < 0.05). We found that there was a significant negative correlation between serum ferritin levels and both total lymphocyte (r = -0.617) and CD3+ lymphocyte (r = -0.718) levels in the control group (p < 0.05). A significant negative correlation was detected between serum ferritin levels and CD3-/CD16+CD56+ NK cell levels in the patient group (r = -0.410) (p < 0.05). CONCLUSION: Splenectomy reduces NK cell levels in patients with ß-TM. The negative relationship between ferritin levels and NK cells indicates that ferritin levels should be kept under control in patients with ß-TM.

Roles of ghrelin, hepcidin and HIF-2α in iron metabolism in iron deficiency anemia.

OBJECTIVES: This study investigates the roles of HIF-2α, hepcidin, and ghrelin in iron deficiency anemia (IDA), the most widespread nutritional disorder globally. MATERIAL AND METHODS: Fifty IDA patients (18-50 years, BMI 19-25) and 40 healthy volunteers were studied. Hemoglobin, ferritin, hepcidin, HIF-2α, and ghrelin levels were analyzed. RESULTS: IDA patients showed lower hemoglobin, ferritin, hepcidin, and ghrelin levels than the control group, but HIF-2α levels were similar. Positive correlations were observed in both groups between hepcidin and HIF-2α (p < 0.001), hepcidin and ghrelin (p < 0.001), and HIF-2α and ghrelin (p < 0.001). Hemoglobin was correlated positively with HIF-2α, and ferritin was correlated positively with HIF-2α in the patient group. CONCLUSION: The study suggests that the low hepcidin levels in IDA patients enhance iron absorption. The lack of significant HIF-2α level differences may be due to the absence of chronic hypoxia in current hemoglobin levels of IDA patients. Moreover, the low ghrelin levels in patients and the correlations between ghrelin, hepcidin, and HIF-2α in both groups indicate their involvement in iron metabolism.

Heterozygote Hemoglobin G-Coushatta as the Cause of a Falsely Decreased Hemoglobin A1C in an Ion-exchange HPLC Method.

Glycated hemoglobin (HbA1c) is used for the assessment of glycemic control in patients with diabetes. The presence of genetic variants of hemoglobin can profoundly affect the accuracy of HbA1c measurement. Here, we report two cases of Hemoglobin G-Coushatta (HBB:c.68A>C) variant that interferes in the measurement of HbA1c by a cation-exchange HPLC (CE-HPLC) method. HbA1c was measured by a CE-HPLC method in a Tosoh HLC-723 G7 instrument. The HbA1c levels were 2.9% and 4%. These results alerted us to a possible presence of hemoglobinopathy. In the hemoglobin variant analysis, HbA2 levels were detected as 78.3% and 40.7% by HPLC using the short program for the Biorad Variant II. HbA1c levels were measured by an immunoturbidimetric assay in a Siemens Dimension instrument. HbA1c levels were reported as 5.5% and 5.3%. DNA mutation analysis was performed to detect the abnormal hemoglobin variant. Presence of Hemoglobin G-Coushatta variant was detected in the patients. The Hb G-Coushatta variants have an impact on the determination of glycated hemoglobin levels using CEHPLC resulting in a false low value. Therefore, it is necessary to use another measurement method.

Effect of iron overload on endocrinopathies in patients with beta-thalassaemia major and intermedia.

INTRODUCTION: Iron overload is a major problem in patients with b-thalassemia major, and it has many structural and metabolic consequences. In this study, we aimed to consider the prevalence of endocrine abnormalities in patients with ß-thalassemia major and thalassemia intermedia. MATERIALS AND METHODS: We ordered following tests for consideration endocrine abnormalities: fasting plasma glucose, oral glucose tolerance, iron, total iron binding capacity, ferritin, thyroid-stimulating hormone (TSH), free thyroxin (fT4), free triiodothyronine (fT3), parathyroid hormone levels. RESULTS: According to our study including 70 patients with thalassemia major, 7 (10%) had diabetes, 5 (7.1%) had impaired glucose tolerance, 9 (12.8%) had hypothyroidism, 2 (2.8%) had hypoparathyroidism, 2 (2.8%) had hyperparathyroidism. Of 22 patients with thalassemia intermedia, 1 (4.5%) had diabetes. CONCLUSIONS: These findings reinforce the importance of regular follow-up of patients with b-thalassemia major and thalassemia intermedia for early detection and management of associated complications. In this way, the prevalence of endocrine abnormalities can be decreased in future.