scholarly journals Correlation between Pancreatic MRI T2* and Iron Overload in Adult Transfusion Dependent Beta Thalassemia Patients with Growth Retardation: A Single Centre Study in Indonesia

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4907-4907
Author(s):  
Faizal Drissa Hasibuan ◽  
Tubagus Djumhana Atmakusuma
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Growth Retardation ◽  
Transferrin Saturation ◽  
Endocrine Function ◽  
Beta Thalassemia ◽  
Endocrine Disorders ◽  
Cross Sectional ◽  
Excess Iron ◽  
Iron Load

Abstract Correlation Between Pancreatic MRI T2* And Iron Overload in Adult Transfusion Dependent Beta Thalassemia Patients With Growth Retardation : A Single Centre Study in Indonesia Faizal Drissa Hasibuan , MD 1,2 , Tb. Djumhana Atmakusuma , MD, PhD 3, 4 1Department of Internal Medicine, 2Faculty of Medicine Yarsi University Jakarta, Indonesia, 3Medical Hematology - Oncology Division of Internal Medicine Department Cipto Mangunkusumo Hospital, 4Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia Correspondence: [email protected] phone +6281533197733 The prevalence of thalassemia in Indonesia is one of the highest in the world. It is estimated that the prevalence of beta thalassemia carrier is around 3-10%. In 2016, thalassemia center in Cipto Mangunkusumo Hospital (RSCM) Jakarta recorded 9031 patients suffering from major thalassemia in Indonesia. 441 are adult thalassemia patients (age 18 and above) recorded in Kiara thalassemia and hematology-oncology clinics in RSCM. Based on a survey from TIF, endocrine aspect of the thalassemia patients is often ignored by the clinicians. Growth retarded patients are commonly found in the thalassemia clinic in RSCM. Publication regarding the pancreas and its correlation with iron overload in adult beta TDT patients is currently not available in Indonesia. Therefore, the aim of this study was to describe iron overload condition based on the pancreatic MRI T2* and its correlation with beta TDT adult patients who suffer from growth retardation. A cross sectional study was conducted to determine the prevalence of endocrine disorders in adult TDT beta patients, followed by looking for correlation of excess iron load with endocrine function in adult TDT beta patients with growth retardation in adult Thalassemia clinic RSCM Jakarta on December 2017. Patients with HBsAg or Anti HCV positive were excluded. Excess iron is defined as Transferin Saturation (ST) greater than 50% regardless of serum ferritin or serum ferritin (FS) levels greater than 1000 ng/mL regardless of ST or both.The growth retardation is defined as the standing height of the research subject which is lower than the Mid Parental Height (MPH) value of both parents. Pancreatic MRI T2* used magneto avanto Siemens 1,5T with CMR software. We found from 58 patients who followed the study, 32 patients underwent the pancreatic MRI T2* examination with 13 female (40,6%) and 19 male (59,4%). Patients with homozygous beta thalassemia are 16 people (50%) and beta/HbE thalassemia 16 people (50%). The Proportion of low pancreatic MRI T2* values was found to be 87.5%, with moderate hemosiderosis in 13 patients (40.63%) and severe hemosiderosis not found (Table 2). The age range of the study subjects was relatively young with a median age of 21 years. Although the median body weight of study subjects was 42 kg, the median BMI still included in normal range. Excess iron content in this study was assessed with serum ferritin, obtained median 4982.5 ng/mL and transferin saturation with a median of 100%, indicates the subject of research are in a state of excess iron load. This is due to the possibility of inflammation, inadequate use of chelation, hemolysis in thalassemia, hypertransfusion to achieve the target of 12 g/dL for women and 13 g/dL for men. There was no significant correlation between serum ferritin and pancreatic MRI T2* value, nor did a significant correlation between transferrin saturation with pancreatic MRI T2* value (Table 3). In this study, there was a high proportion of subjects with low pancreatic MRI T2* value of 28 subjects (87,5%), divided into 15 mild hemosiderosis (46,87%), 13 moderate hemosiderosis (40,62%) and none of severe hemosiderosis. Our study is the first study which look for the correlation of the excess iron load (serum ferritin and transferrin saturation) with endocrine function in adult TDT beta patients with retardation of growth in Indonesia. This research has limitations. First, it was a cross sectional study so it is not known exactly the beginning of endocrine disorders in the subject . The second limitation, analysis of iron chelation therapy did not do in this study. Finally, we concluded that there was no correlation between pancreatic MRI T2* and iron overload based on serum ferritin and transferrin saturation. Further longitudinal studies in adult TDT patients with thalassemia who have not and have retarded growth were needed. Disclosures No relevant conflicts of interest to declare.

scholarly journals Correlations between Iron Load and CD4 in Adult Transfusion-Dependent Beta Thalassemia

Anemia ◽  
2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Tubagus Djumhana Atmakusuma ◽  
Ralph Girson ◽  
Sukamto Koesnoe
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Transferrin Saturation ◽  
Red Blood Cell Transfusion ◽  
Beta Thalassemia ◽  
Cross Sectional ◽  
X Ray ◽  
Chest X Ray ◽  
Anti Hiv ◽  
Insignificant Correlation

Background. Thalassemia is a hereditary disease, and severe anemia is the main phenotype of major thalassemia. Furthermore, the most important method in the management of this disease is red blood cell transfusion. Regular transfusions administered 1 or 2 times every month improve prognosis and survival. However, there is higher risk of infections and iron overload, especially in transfusion-dependent thalassemia (TDT). Infections are the second leading cause of death in adult TDT, after heart failure. Higher risk of infection is also influenced by multiple blood transfusions which causes alteration in immune response due to alloimmunization, transfusion-related infections, and iron overload. Meanwhile, iron overload in TDT alters both innate and specific immune responses. Furthermore, previous studies have shown the correlation between ferritin with CD4, but this has not been carried out in Indonesia. Therefore, this study aims to determine the correlations between iron overload (serum ferritin and transferrin saturation) and specific immune cells (CD4). Methods. This is a cross-sectional study, and a total number of 64 subjects were examined consecutively. Chest X-ray and blood sera were obtained. The total number of subjects was 64. The seromarkers HBsAg, anti-HCV, and anti-HIV were tested using the ELISA method. Serum ferritin and transferrin saturation was tested using ECLIA, and lymphocyte subsets were analyzed using flowcytometry. Meanwhile, the correlation between variables was determined using Spearman’s test. Results. The results showed that 4.9% subjects were HBsAg positive, 10.7% were anti-HCV positive, and none were anti-HIV positive. There were 4 subjects with lung tuberculosis based on the 41 chest X-ray. Meanwhile, there was a weak negative and insignificant correlation between serum ferritin with CD4 ( p = 0.75 ; r = −0.04) and a weak positive and insignificant correlation between transferrin saturation with CD4 ( p = 0.133 ; r = 0.19). Conclusion. There were no correlations between iron overload (ferritin) and cellular immunity (CD4) in adult transfusion-dependent thalassemia.

scholarly journals Liver Enzymes in Children with beta-Thalassemia Major: Correlation with Iron Overload and Viral Hepatitis

2015 ◽  
Vol 3 (2) ◽  
pp. 287-292 ◽  
Author(s):  
Khaled M. Salama ◽  
Ola M. Ibrahim ◽  
Ahmed M. Kaddah ◽  
Samia Boseila ◽  
Leila Abu Ismail ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Iron Overload ◽  
Viral Hepatitis ◽  
Serum Ferritin ◽  
Liver Enzymes ◽  
Transferrin Saturation ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Elevated Liver Enzymes ◽  
Hcv Antibody

BACKGROUND: Beta Thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%) with an estimated carrier rate of 9-10.2%. Injury to the liver, whether acute or chronic, eventually results in an increase in serum concentrations of Alanine transaminase (ALT) and Aspartate transaminase (AST).AIM: Evaluating the potentiating effect of iron overload & viral hepatitis infection on the liver enzymes.PATIENTS AND METHODS: Eighty (80) thalassemia major patients were studied with respect to liver enzymes, ferritin, transferrin saturation, HBsAg, anti-HCV antibody and HCV-PCR for anti-HCV positive patients.RESULTS: Fifty % of the patients were anti-HCV positive and 55% of them were HCV-PCR positive. Patients with elevated ALT and AST levels had significantly higher mean serum ferritin than those with normal levels. Anti-HCV positive patients had higher mean serum ferritin, serum ALT, AST and GGT levels and higher age and duration of blood transfusion than the negative group. HCV-PCR positive patients had higher mean serum ferritin and serum ALT and also higher age and duration of blood transfusion than the negative group.CONCLUSION: Iron overload is a main leading cause of elevated liver enzymes, and presence of HCV infection is significantly related to the increased iron overload.

Disease Specific Modulation of Serum Hepcidin: Impact of GDF-15 and Iron Metabolism Markers in Thalassemia Major, Thalassemia Intermedia and Sickle Cell Disease: A Univariate and Multivariate Analysis.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3850-3850 ◽  
Author(s):  
Farzana Sayani ◽  
Sukhvinder Bansal ◽  
Patricia Evans ◽  
Aalim Weljie ◽  
Robert C Hider ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Iron ◽  
Transferrin Saturation ◽  
Thalassemia Major ◽  
Thalassemia Intermedia ◽  
Iron Load ◽  
Serum Hepcidin ◽  
Disease Specific

Abstract Background. Factors that determine net synthesis of hepcidin and hence iron absorption and distribution depend on a balance of competing factors which may be disease specific. Such factors include anemia, ineffective erythropoiesis (IE), transferrin saturation (Tf sat), iron overload and inflammation. Recently GDF-15, a marker of erythroid maturation and hence IE, has been linked with depression of hepcidin synthesis in vitro and showed elevated levels in beta thalassemia (Tanno et al, Nat Med, 2007). The relationship of hepcidin synthesis to iron overload in sickle cell disease (SCD) is not clear and may differ from thalassemia syndromes because IE is less marked. We wished to establish whether the dominant factors determining net hepcidin synthesis differed between patients with SCD and those with thalassemia intermedia (TI) and thalassemia major (TM). Patients and methods. Serum hepcidin was measured in hypertransfused (Hb>9.5g/dl) patients with TM (n=18), untransfused or sporadically transfused patients with thalassemia intermedia TI (n=18), and multi-transfused patients with SCD (n=24), and related to markers of anemia, iron overload and erythroid expansion. A newly developed mass spectrometry assay (Bansal et al, Anal Biochem, 2008, In Press) was used to determine serum hepcidin. GDF-15 was measured by an ELISA assay. Multivariate analysis was performed using SIMCA-P software and partial least squares for discriminant analysis (PLS-DA), using samples from each of the clinical groups to investigate relationships between hepcidin, serum iron, non-transferrin bound iron (NTBI), transferrin saturation (Tf sat), serum ferritin, liver iron, transfusion history, erythropoietin, hemoglobin and GDF-15. Results. Serum hepcidin levels were higher in TM (13.9 ± 10.0 nmol/L) than SCD (8.51±8.16 nmol/L, p=0.043) whereas values in TI (3.82 ±3.56 nmol/L) were close to healthy controls (4.04 ± 2.06nmol/l). However, when SCD patients were matched for levels of anemia and iron load with TM, plasma hepcidin levels were similar or higher in SCD. GDF-15 values were highest for TI (11,444± 2177 ng/l), than TM (4117 ± 577 ng/l, P<0.001), whilst SCD patients had the lowest values (1227 ± 208 ng/l, P<0.001 vs TM). Univariate analysis in all patients grouped together showed positive correlations of hepcidin with serum ferritin (r=0.55, p <0.0001) and level of anemia (r=0.27, p= 0.045). Disease specific relationships were identified: negative correlations of serum hepcidin with Tf sat (r=−0.43) and NTBI (r=−0.45) were found for TI and TM but not in SCD, whereas ferritin showed a positive correlation in TM and SCD (r=0.51 and r= 0.56) but not in TI. GDF-15 correlated negatively with hepcidin in TI (r=0.51) but showed no relationship in SCD or TM. Positive correlations of GDF-15 with markers of plasma iron metabolism were seen in TI such as serum iron (r= 0.56), NTBI (r=0.45) and transferrin saturation (r=0.45). These were not seen in TM and tended to be negative relationships (r= −0.45, r= 0.25, r=0.59 respectively). In multivariate analysis, the variables responsible for the separation of the 3 patient groups clustered in 3 major categories including iron handling (serum iron, transferrin saturation, NTBI), ineffective erythropoiesis (GDF-15) and iron loading (ferritin, transfusion history). Hepcidin co-clustered with the iron loading group and was inversely correlated with GDF-15. Conclusion. Competing regulatory effects on hepcidin synthesis differ between TM, TI and SCD. In TI, hepcidin synthesis is suppressed by IE as shown by a dominant effect of GDF-15. In TM, GDF-15 effects on plasma hepcidin are less marked, as IE is lower due to hypertransfusion. This difference is particularly striking in patients at UCLH due to the divergent transfusion policies between TI and TM. The dominant modulating factors in TM are positive relationships to iron load (serum ferritin) but negative relationship with NTBI, serum iron and Tf saturation. However it is not yet clear whether the relationship of NTBI to hepcidin implies direct negative regulatory effect. In multi-transfused SCD patients, GDF-15 (IE) and NTBI have insignificant relationships to plasma hepcidin, with iron load (ferritin) showing the dominant effect: other effects in SCD such as those of chronic inflammation were not examined but require further investigation.

Investigation of Gonadal Function, Puberty, and their relationship to Serum Ferritin in Male patients with β-Thalassemia major in Syria

2021 ◽  
pp. 3595-3602
Author(s):  
Roula Shakkour ◽  
Taghrid Hammoud ◽  
Yasser Mukhalalaty ◽  
Faizeh Al Quobaili
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
Delayed Puberty ◽  
Beta Thalassemia ◽  
Endocrine Disorders ◽  
Gonadal Function ◽  
Pubertal Status ◽  
Gonadal Dysfunction ◽  
Male Patients

Objectives: Endocrine disorders continue to affect the health of thalassemia patients, foremost of which is hypogonadism being the most frequent endocrine complication that involves 70-80% of beta-thalassemia major (β-TM) patients. Actually, the role of iron overload in endocrine complications is well known. Our study goals were to investigate gonadal function, assess pubertal status among Syrian male patients with β-TM and correlate hormonal panel with serum ferritin as the marker of iron overload. Methods: 56 β-TM regularly transfused male patients were enrolled in this study, they were 21.91±5.01 years old. FSH, LH, Total Testosterone, and Serum Ferritin were measured for all patients, 52 of them undergone pubertal status evaluation. Results: Results showed that 60.7% of patients suffered from hypogonadism, which was hypogonadotropic hypogonadism in 97.06% of them. Delayed puberty was seen in 7.7% of the patients, while arrested puberty was found in 82.69% of them. All patients had iron overload and 92.86% of them suffered from severe iron elevation. Both gonadal and pubertal status were independent of the serum ferritin levels (P=0.73), (P=0.81) respectively. There was significant positive correlation between FSH: LH (r=0.584, P=0.0001), FSH: Testosterone (r=0.562, P=0.0001), LH: Testosterone (r=0.746, P=0.0001), MCHC: Testosterone (r=0.292, P=0.038), and BMI: Hb (r=0.351, P=0.009). Conclusions: Our findings indicated that hypogonadism, arrested puberty and severe iron overload were highly prevalent among male patients with β-TM. Patients with better gonadal reserve have higher BMI than those with gonadal dysfunction. We suggest that hypogonadism in β-TM patients is not directly related to serum ferritin levels; other potential factors (such as chronic anemia, hypoxia, and genetic predisposition) may contribute. Also we suggest that adequate blood transfusion and appropriate iron chelation, along with regular evaluation for gonadal status and timely intervention can improve the management of aforementioned complications, thus ameliorating patients’ quality of life.

scholarly journals Free testosterone level in patients with homozygous beta thalassemia on regular transfusions regimen

2016 ◽  
Vol 44 (2) ◽  
pp. 73
Author(s):  
Riadi Wirawan ◽  
Elly Santosa ◽  
Lyana Setiawan ◽  
Bulan Ginting Munthe ◽  
Dalima AW Astrawinata
Keyword(s):  
Testosterone Level ◽  
Reference Range ◽  
Transferrin Saturation ◽  
Free Testosterone ◽  
Cross Sectional Study ◽  
Endocrine Function ◽  
Beta Thalassemia ◽  
Cross Sectional ◽  
Significant Difference ◽  
Free Testosterone Level

Background Patients with homozygous beta thalassemia requireregular transfusions which will lead to iron deposition in tissuesincluding testicles.Objective This study aimed to evaluate testicular function in syn-thesizing testosterone by measuring free testosterone level. Thecorrelation between free testosterone level and transferrin satura-tion was evaluated.Methods This was a cross sectional study. Sampling was doneconsecutively. Free testosterone level was measured by radioim-munoassay in 20 homozygous beta thalassemic patients receiv-ing regular transfusions and compared to 20 healthy subjects.Results Fourteen out of 20 patients showed free testosterone levelbelow the reference range. Out of 20 thalassemic patients, onepatient’s serum was not enough for transferrin saturation determi-nation. Among the 19 patients, 18 had transferrin saturation above55%, 1 less than 55%, while all healthy subjects had normal trans-ferrin saturation. A significant difference was found between thetwo groups, both in the free testosterone level (p=0.001) and trans-ferrin saturation (p<0.001). A very weak correlation was found be-tween free testosterone level and transferrin saturation (r=-0.215).Conclusion We concluded that there might be a relationship be-tween iron overload and testicular endocrine function in patientswith homozygous beta thalassemia receiving regular transfusions

scholarly journals Pulmonary dysfunction in children with beta thalassemia major in relation with iron overload - a cross sectional hospital based study

2015 ◽  
Vol 6 (5) ◽  
pp. 47-50 ◽  
Author(s):  
A Boddu ◽  
A Kumble ◽  
S Mahalingam ◽  
BS Baliga ◽  
B Achappa
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
P Value ◽  
Pulmonary Dysfunction ◽  
Cross Sectional ◽  
Transfusion Therapy ◽  
Respiratory Dysfunction ◽  
Respiratory Impairment

Background: Previously many patients with thalassemia major died from severe anemia during first decade, but with modern transfusion therapy many now survive beyond childhood. Because of such therapy, however excessive iron deposition occurs in lungs and causing respiratory dysfunction. Respiratory impairment occurs because of defective chelation and repeated transfusions. Objectives: To a) study pattern of respiratory impairment using spirometry, b) estimate iron overload by measuring serum ferritin levels, c) correlate a&b. Methodology:Thalassemia children >7year, on regular blood transfusion were included in the study after getting institutional ethical clearance .Standardised pulmonary function test was done using spirometry. Iron overload was assessed using serum ferritin levels. Severity of pulmonary dysfunction was correlated with serum ferritin levels. Results:Total of 42 children were included 62% were males and 38% were females (with median age 12yrs). By spirometry 95% had restrictive pattern of respiratory dysfunction. Mean ferritin value was 4152. Out of them10 (23.8%) mild, 25(59%) moderate and 5(12%) severe dysfunction based on FEV1 and FVC. The mean ferritin values in severe respiratory dysfunction is 6275 which is significantly higher when compared to moderate (4249) and mild (3066) pattern of respiratory dysfunction. None of the children had evidence of CCF. Significant correlation (p value=0.003) was found between severity of pulmonary dysfunction with ferritin values and also with weight of the child (p value=0.007). No other significant correlation found between severity pattern and transfusion index, age or height. Conclusion:Restrictive pattern is most common pulmonary dysfunction seen in chronic iron overloaded thalassemia major children. Regular blood transfusions with adequate chelation decrease incidence of pulmonary dysfunction. Screening of all thalassemia children using spirometry is need of the hour. DOI: http://dx.doi.org/10.3126/ajms.v6i5.11782Asian Journal of Medical Sciences Vol.6(5) 2015 47-50

scholarly journals GROWTH AND ENDOCRINE FUNCTION IN TUNISIAN THALASSEMIA MAJOR PATIENTS

2018 ◽  
Vol 10 (1) ◽  
pp. 2018031 ◽  
Author(s):  
Naouel GUIRAT
Keyword(s):  
Iron Overload ◽  
Thalassemia Major ◽  
Early Years ◽  
Endocrine Function ◽  
Delayed Puberty ◽  
Beta Thalassemia ◽  
Endocrine Disorders ◽  
Anthropometric Parameters ◽  
Ferritin Concentration ◽  
Hereditary Disorders

Beta-thalassemia major (TM) is among prevalent hereditary disorders imposing high expenses on health-care system worldwide. The patient’s survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity on various organs including endocrine glands. This article provides an overview of  endocrine disorders in beta-TM patients. This single center investigation enrolled 28 beta-TM patients (16 males, 12 females)  regularly transfused with packed red cell since early years of life. For each patient were determined: age, sex, number of transfusions received, history of splenectomy and anthropometric parameters. Evaluation for hormonal status including growth, gonadal, thyroid, adrenal cortex, and parathyroid glands was done for all patients. Dual energy X-ray absorptiometry was used to diagnose osteoporosis. Assessment of iron overload status was performed by measuring the serum ferritin concentration and the results of magnetic resonance imaging T2*. Growth retardation was found in 16 of the 28 studied patients (57 %).Thirteen among them had delayed puberty. Spontaneous puberty was achieved in 16 cases. Growth hormone (GH) deficiency was found in 10 cases (35 %). Seventeen among the studied patients (60 %) developed disorders of glucose homeostasis. Subclinical hypothyroidism was found in six patients (21 %). Intensive chelation therapy had allowed the reversibility of this complication in five cases. Adrenal Insufficiency was found in 9 cases (32%). Hypoparathyroidism has occurred in one case. Ten of the 28 studied patients had osteoporosis (35%). Twenty-three of the 28 studied patients (82%) had at least one endocrine complication.

Clinical-Reported Outcomes Of Deferoxamine Versus Deferasirox In The Treatment Of Homozygous BetaThalassemia

2018 ◽  
Vol 9 (SPL1) ◽  
Author(s):  
Zeina A Munim Al-Thanoon ◽  
Zeina A Munim Al-Thanoon ◽  
Mustafa Basil ◽  
Nasih A Al-Kazzaz
Keyword(s):  
Serum Ferritin ◽  
Iron Chelation ◽  
Iron Chelator ◽  
Beta Thalassemia ◽  
Control Group ◽  
Current Standard ◽  
Cross Sectional ◽  
Significant Difference ◽  
Multiple Blood ◽  
Group 2

Iron chelation therapy with deferoxamine (DFO),the current standard for the treatment of iron overload in patients with betathalassemia,requires regular subcutaneous or intravenous infusions. This can lead to reduced quality of life and poor adherence,resulting in increased morbidity and mortality in iron-overloaded patients with beta-thalassemia. Deferasirox (DFX) is an orally administered iron chelator that has been approved for use in many countries. The requirement of an effective,well tolerated iron chelator with a less demanding mode of administration has led to the development of deferasirox. The present study was aimed to compare the satisfaction and compliance with deferoxamine versus deferasirox (Exjade®),a novel oral iron chelator in patients with transfusion - dependent beta- thalassemia. A cross-sectional,single-center investigation study was carried out in the Thalassemia Center of Ibn-Atheer Teaching Hospital in Nineveh province,Iraq. One hundred and eight thalassemic patients aged between 2- 20 years old having received multiple blood transfusions and a serum ferritin greater than 1500 ng/ml. Patients were randomised into two groups. Group 1 received deferoxamine at a dose of 20-50mg/kg/day and group 2 received deferasirox at the dose of 10-30 mg/kg/day. Another 56 apparently healthy volunteers were used as a control group. The assessment of chelation was done during the period between November 2013 and February 2014 by measurement of serum ferritin. Satisfaction and compliance was assessed by using a special questionnaire prepared by the researcher. Out of the 108 thalassemic patients enrolled there was no discontinuation in treatment with the two drugs under study. The serum ferritin did not change significantly in any of the chelation groups. In comparison with the patients who were treated with DFO,those receiving DFX reported a significantly higher rate of compliance and satisfaction (P < 0.05). However,no significant difference was observed between the two groups regarding their satisfaction (P > 0.05).Compliance with deferasirox (50 %) was more than that with deferoxamine (20 %). Satisfaction with deferoxamine was significantly lower than deferasirox (p= 0.00).

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