الفهرس 
Disorders Affecting HemoglobinOnly 14 pages are availabe for public view
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Abstract
Background
Beta Thalassemia is disease characterized by quantitative defect in globin chain production in hemoglobin, leading to microcytic hemolytic anemia as well as progressive iron overload due to blood transfusions as well as increased iron absorption in the intestines. Iron overload mediates much of the morbidity and mortality in beta Thalassemia. Current markers of measuring iron overload such as serum ferritin and MRI imaging to detect iron overload in the liver and heart are available but suffer from drawbacks such as the lack of specificity of serum ferritin and the cost and inconvenience of performing MRI. New methods are needed for evaluating iron overload. Hepcidin is the master hormone is charge of iron metabolism. Serum hepcidin levels and its correlation with other markers of iron overload and MRI findings were assessed in the beta Thalassemia patients.
Aim of work
The aim of the work was to correlate serum hepcidin level in patients with beta thalassemia major and beta thalassemia intermediate to serum ferritin and MRI T2* findings in the liver and heart. This was done to assess the utility of using serum hepcidin in assessing iron overload and whether it could be used to supplement or replace other traditional methods of assessing iron overload in the future.
Patient and method
This study involved 30 patients and 10 controls. The patients were selected from the Hematology department of Ain Shams University Hospital from the outpatient clinic from January 2017 until August 2017. Hepcidin level was assayed quantitatively using a sandwich ELISA detection kit. The sample type used was serum. The samples were collected in the early morning from 8 – 9:30 am as a pre-transfusion sample. Patients also performed labs including serum ferritin, CBC, TSH, HCV Ab, HBsAg, AST, ALT. Patients also performed cardiac and liver MRI T2* to check for iron overload.
Results
The patient population included 30 patients with beta thalassemia major and intermediate. The median age of the patients was 31 years old. The patients consisted of 7 patients with thalassemia major (TM) and 23 patients with thalassemia intermediate (TI). As a group, they shared several characteristics; First, the patients studied were all on regular blood transfusion schedules, with the least patient receiving 12 PRBCs/year and the median patient receiving 21 PRBCs/year. Second, as a group, the patients were heavily iron overloaded, with the median ferritin among the patient population being 2000 ng/dL and all patients showing some degree of liver iron overload according to MRI T2* findings.
Serum hepcidin was correlated with serum ferritin levels and MRI T2* findings for liver and iron overload. A significant correlation was not found between the two, which was consistent with other studies that showed that iron overload in states of ineffective erythropoiesis characterized by simultaneous iron overload and anemia was not the prime factor influencing serum hepcidin level. However, a significant correlation was found between serum hepcidin level and gender, being significantly lower in males than females. A study done by Parsricha et al demonstrated the same findings that males had significantly lower hepcidin levels than females (7). The authors of the study went on to explain that due to the “androgen effects on erythropoiesis, men may thus require higher hemoglobin concentrations to suppress erythropoietic drive.”
Significant correlation was also found between hepcidin and anemia, which corresponded to other studies that produced the same results. This is due to the erythropoietic drive initiated by anemia which leads to suppressed hepcidin levels. Significant correlation was also found between serum hepcidin level and chelators, with higher hepcidin levels encountered in patients on deferoxamine and desferasirox compared to patients on deferiprone.
Conclusion
While hepcidin has a great therapeutic and diagnostic potential in patients with anemia, it is not a sensitive marker in iron overload in patients with beta thalassemia major and intermediate. This is due to the effect of competing factors on serum hepcidin levels (anemia, erythropoietic drive, iron overload). Dedicated studies can be done in the future to clarify the effect of chelator therapy on hepcidin levels and the correlation between HCV or HBV infection in beta Thalassemia patients to serum hepcidin levels. Hepcidin is the master hormone controlling iron metabolism and has exciting potential as both a diagnostic and therapeutic marker in anemias.