A Prospective Study of Thyroid Function Status in Patients of Haemoglobin E Beta Thalassemia and Correlation with Serum Ferritin Level

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4837-4837
Author(s):  
Tuphan Kanti Dolai ◽  
Shuvra Neel Baul ◽  
Prakas Kumar Mandal ◽  
Rajib De ◽  
Prantar Chakrabarti

Abstract Introduction Haemoglobin E-beta-thalassaemia (EBT) represent approximately 50 per cent of those affected with severe beta thalassemia.The highest frequencies are observed in India, Bangladesh and throughout Southeast Asia. Endocrinopathies are now amongst the common complications of thalassaemia and it is multifactorial in origin. Iron overload in EBT is also multifactorial. This study was undertaken to evaluate the thyroid dysfunction in patients of EBT and its correlation with serum ferritin levels. Methods EBT patients were evaluated prospectively to assess thyroid dysfunction status and correlate it with serum ferritin levels. High performance liquid chromatography was performed with Bio-rad beta thalassaemia short program variant II. Serum ferritin estimation was done with microplate immunoenzymometric assay and thyroid assay for TSH, free T4 and T3 were done by access 2 Immunoassay System, Beckman Coulter. Results 50 patients with EBT were evaluated. The mean age of patients were 19.7 years (range: 12-47). There were 28 males and 22 females. There were 41(82%) and 9(18%) transfusion dependent and transfusion independent patients respectively and 40(80%) were on chelation therapy. In this cohort 22(44%) patients had thyroid dysfunction. Six (12%) and 16(32%) of patients were having hypothyroidism and subclinical hypothyroidism respectively. Mean ± Standard deviation (S.D) of serum ferritin level with hypothyroidism, subclinical hypothyroidism and euthyroidism was 1077 ± 371.8 ng/ml ,1422 ± 1361.0 ng/ml and 1252 ± 664.4 ng/ml respectively with correlation coefficient =0 [Fig 1 and Fig 2]. Serum ferritin levels do not predict thyroid dysfunction in patients of EBT. Male and female have equal preponderance for thyroid dysfunction. Patients with subclinical hypothyroidism were having few symptoms compared to frank hypothyroidism and majority of symptoms in either scenario were masked by thalassemia itself. Earlier age of onset of EBT is associated more with thyroid dysfunction however the association is not statistically significant (p=0.2). There was no association between spleen size and thyroid dysfunction (p=0.7). Conclusions Thyroid dysfunction was seen in 42% of EBT patients. Prevalence of hypothyroidism was found to be higher in EBT patients compared to general population but a definite correlation with the serum ferritin levels could not be established. Figure 1 Mean serum ferritin level and thyroid status Figure 1. Mean serum ferritin level and thyroid status Figure 2 Correlation of serum TSH and ferritin level (correlation coefficient =0) Figure 2. Correlation of serum TSH and ferritin level (correlation coefficient =0) Disclosures No relevant conflicts of interest to declare.

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4819-4819
Author(s):  
Natthapat Rujeerapaiboon ◽  
Adisak Tantiworawit ◽  
Pokpong Piriyakhuntorn ◽  
Thanawat Rattanathammethee ◽  
Sasinee Hantrakool ◽  
...  

Background: Serum ferritin is widely used as a marker of iron overload in thalassemia patients. However, the ferritin level is affected by active infections or inflammation. The association between viral hepatitis and serum ferritin level in thalassemia patients is still unclear. This study aimed to determine the effect of chronic viral hepatitis on serum ferritin level in thalassemia patients. Methods: This was a cross-sectional study in thalassemia patients aged ≥15 years-old at Chiang Mai University hospital. We expected that thalassemic patients in our clinic have a mean serum ferritin of 767 ng/mL with a standard deviation of 210 ng/mL. As a result, we have to enroll a total of 28 patients to demonstrate 30% difference of mean serum ferritin when the power was set at 80% with alpha level of 0.05. Information on chronic viral hepatitis, mean serum ferritin and liver iron concentration (LIC) as measured by T2* MRI were collected. Chronic viral hepatitis status was confirmed by either HBV DNA or HCV RNA testing. Patients were categorized to hepatitis and non-hepatitis group. Serum ferritin levels were compared between two groups. LIC measurement was used as a gold standard for iron overload. Subgroup analysis was performed according to iron overload and transfusion requirement status. Categorical and continuous variables were compared using the Chi-squared test and T-test, respectively. The correlation between viral loads and mean serum ferritin levels was analyzed by Pearson's correlation. Result: Of 32 thalassemia patients (25 non-transfusion dependent [NTDT] and 7 transfusion dependents [TDT]), 13 patients had chronic viral hepatitis (7 with hepatitis B and 6 with hepatitis C infections). The LIC between hepatitis and non-hepatitis groups were not significantly different (7.28 [SD 4.7] vs 9.08 [SD 5.2] mg Fe/g, p=0.19). In the higher LIC group (≥ 5 mg Fe/g), the mean serum ferritin level was higher in the hepatitis group than non-hepatitis group (1,776 [SD 488] vs 967 [SD 860] ng/mL, p=0.03). For the lower LIC group (<5 mg Fe/g), the mean ferritin levels were not significantly different between the hepatitis and non-hepatitis groups (646 [SD 224] vs 459 [SD 205] ng/mL, p=0.22). The correlation between the viral load and mean ferritin level in NTDT group showed a significant linear correlation with R=0.7 (p=0.04). Conclusions: We observe a higher serum ferritin level among thalassemia patients who concurrently have chronic viral hepatitis. Chronic viral hepatitis is a possible cause of a falsely high ferritin level in these patient population. Furthermore, the viral load is positively correlated with serum ferritin level. Disclosures No relevant conflicts of interest to declare.


2020 ◽  
Vol 4 (1) ◽  
pp. 16-18
Author(s):  
Khaza Amirul Islam ◽  
Md. Shafiul Azam ◽  
Nishat Mahzabin ◽  
Md. Rafiquzzaman Khan ◽  
Md. Salahuddin Shah ◽  
...  

The aim of the study was to determine the frequency of hypothyroidism in transfusion dependent thalassaemia patients. A total of 64 transfusion dependent thalassaemia (TDT) patients were included in this cross-sectional study from April, 2018 to September, 2019 according to selection criteria. Thyroid hormone status was assessed by estimation of serum FT4 and serum TSH. Body iron load was estimated by serum ferritin level. The study sample consisted of 43 male and 21 female TDT patients, with a mean age of 25.5 years. Total 28% patients were found hypothyroid, 11% were overt hypothyroid and 17% were subclinical hypothyroid. Mean serum ferritin level was 2462.6 ng/ml. Significant correlation was not found between serum TSH and Serum ferritin level (p = 0.055). Total unit of transfusion (p=0.001) and duration of transfusion therapy (p=0.003) were significantly associated with development of hypothyroidism.


2021 ◽  
Vol 9 (B) ◽  
pp. 1405-1409
Author(s):  
Ahmed I. I. Ansaf ◽  
Safa Faraj ◽  
Hussien A. Abdul-Azziz

Background: Hypothyroidism is one of the commonest endocrine complications that can happen in patients with Beta-Thalassemia Major (BTM) above nine years old which may pass hidden and untreated for years.   Aim: To evaluate the thyroid status and types of thyroid dysfunction in Beta-Thalassemia Major Patients above 9 years of age. Method: A Cross-Sectional study was performed on sixty-six randomly selected patients above 12 years of age with BTM in Al-Kut Heriditory Blood Disease Center at Wassit province from March to August 2020. Thyroid function tests were done (TSH, T3, and T4) to detect the thyroid status and identify patients with thyroid dysfunction and determine the type of hypothyroidism (Primary, central, Subclinical). Data collected include Age, Sex, weight, height, Serum ferritin and mean hemoglobin level, Type of chelation therapy, dose, compliance, and frequency of blood transfusion, other diseases such as Diabetes Mellitus, HCV, HBV, HIV, and Heart Failure were evaluated statistically. Results:   In the studied patients, Thirty-one patients (46.96%) were found to have hypothyroidism;( Seven of them (10.6%) had primary hypothyroidism, Sixteen(24.2%) had subclinical hypothyroidism and eight (12.1%) had central hypothyroidism).  Twenty-one (67.74 %) patients with Hypothyroidism were found to have stunted height or short stature (with significant p.value 0.03) from the 13 (41.9%) who had subclinical hypothyroidism.  By Pearson correlation, increasing serum ferritin levels significantly affect decreasing thyroxin (T4) levels with inverse correlation (P value 0.0001), (r)= -0.45. Conclusion:  in this study, there was a high prevalence of hypothyroidism in BTM and subclinical hypothyroidism is the most common type, which signifies the importance of regular screening and close supervision especially when high ferritin and or short stature are present.        


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4848-4848
Author(s):  
Jadwiga Dwilewicz-Trojaczek ◽  
Krzysztof Madry ◽  
Beata Stella-Holowiecka ◽  
Andrzej Mital ◽  
Anna Szmigielska ◽  
...  

Abstract Abstract 4848 Background Myelodysplastic syndrome(MDS) belong to the most common hematological diseases however epidemiological data on MDS are sparse. Until 2008 there were no data about epidemiology of MDS in Poland. Methods From 03.2008-05.2009 we have registered 966 patients in Polish MDS Registry. We have included only alive patients of various time of diagnosis. Patients from 22 centers were diagnosed according to WHO 2001 criteria. Results There were 508(53%)males and 458(47%) females. Median age at diagnosis was 70(range 19-99). Under 50 were 83(9%) cases with preponderance of females- 51 cases( males 32cases), between 50-70 there were 353(41%) cases, half of the patients-432(50%) were above 70( 247 males and 185 females).Prior chemotherapy and/or radiotherapy had 37((3,8%) patients. Distribution of MDS subtypes was as follows: RA-170(20%) cases, RARS-58(7%), RCMD-244(28%), RCMD-RS-18(2%), RAEB-1-120(14%), RAEB-2-169(19,5%), 5q- -40(4,6%), MDS-U-44(5%).In 103(10%) subtype was not done. Karyotype was available in 276(28%) cases. Cytogenetic risk groups were: low risk-182(68%), intermediate-52(20%) and high risk-33(12%). The most frequent cytogenetic results were: normal karyotype 44%, isolated 5q deletion 19%, complex karyotype 6%, 5q deletion + another one change 3% and 5q deletion with at least 2 changes 3%. According to IPSS risk groups low risk was found in 61( 22%) of cases, intermediate-1 -130(48%), intermediate-2-47(17%) and high risk in 31(11,5%). Median values of Hb was 9,1 g/dL, plts 129 G/L, ANC 1,7 G/L. RBC transfusion dependent were 429(44%) patients and platelet transfusion dependent were 100( 11%) pts. At least 2 U/month RBC transfusion requirement was 140(14%) patients. Serum ferritin level was assessed in 530 cases-171 of them( 32%) had higher than 1000μg/L level. Conclusions We have observed predominance of females among MDS patients under 50. Half of the patients had RA or RCMD subtype. Isolated 5 q deletion was the most frequent cytogenetic abnormality. Forty four percentage of patients was RBC transfusion dependant. Serum ferritin level was significantly elevated in 32% of assessed patients at the moment of MDS diagnosis. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2218-2218
Author(s):  
Bora Kim ◽  
Kyung Taek Hong ◽  
Ji Won Lee ◽  
Kyung-Sang Yu ◽  
In-Jin Jang ◽  
...  

Abstract Optimal dosing for busulfan is important for minimization of systemic toxicity from overexposure and graft failure or relapse from underexposure. Herein, we investigated potential markers for predicting individual variation in the pharmacokinetics of busulfan, and suggested possible mechanism for inter-individual variability by using pharmacometabolomics. Fifty-nine pediatric patients undergoing busulfan-based conditioning chemotherapy for hematopoietic stem cell transplantation were divided into three groups according to the area under the concentration-time curve (AUC) of busulfan; low-, medium-, and high-AUC group. Nontargeted metabolic profiling of baseline urine samples showed that deferoxamine metabolites were abundant, while 2 acylcarnitines and phenylacetylglutamine were significantly lower in high-AUC group, compared with low-AUC group. Higher level of deferoxamine, an iron-chelating agent for the patients with a high serum ferritin level during the conditioning chemotherapy, suggested pharmacokinetic interaction between serum ferritin level and busulfan exposure. Retrospective analysis of the correlation between serum ferritin level and busulfan AUC showed positive correlation in 130 pediatric patients. The optimal busulfan dose to meet the target AUC of 18,750 μg*h/L/day was calculated to be 119.7 ± 30.1 mg/m2 in patients with ferritin < 1,000 ng/mL and 106.1 ± 29.3 mg/m2in patients with ferritin ≥ 1,000 ng/mL (P=0.021). Mechanismly, previous studies have indicated that ferritin, acylcarnitine and phenylacetylglutamine are closely associated with liver function. Increased serum ferritin is thought to be responsible for an increased production of oxygen free radicals and activation of GSH turnover, which means reduction of GSH levels in both plasma and erythrocytes and this depletion seems to be related to the decrease of busulfan metabolism. Down regulation of acylcarnitines is associated with deregulation of mitochondrial fatty acid β-oxidation by hepatic injury. PAGN, a marker of waste nitrogen scavenger, was down-regulated which indicates ammonia-related metabolism could also be involved in busulfan exposure. Hyperammonemia, a clinical condition of elevated ammonia levels, is mainly lead by liver cell damage. Taken together, our findings demonstrate that elevated serum ferritin levels are a potential biomarker correlated with busulfan exposure and provide some evidence that busulfan metabolism seems to decrease in the patients with reduced liver function. Disclosures No relevant conflicts of interest to declare.


Author(s):  
Babaeva T.N. ◽  
Seregina O.B. ◽  
Pospelova T.I.

At present, the serum ferritin level is not included in the list of prognostic factors; however, it is known that its increased serum level in patients with malignant neoplasms relates with the tumor burden, the degree of disease activity and correlates with a worse prognosis in patients with hematologic malignancies.The normalization of serum ferritin level during remission period confirms the involving of hyperferritinemia in mechanisms of tumor progression and may testify for clinical importance of measurement of serum ferritin level in patients, including those with malignant lymphomas. Objective:The aim of this study was to assess of the prognostic significance of high ferritin levels at the onset of the disease in patients with malignant lymphomas. Materials and methods:98 patients with malignant lymphomaswere enrolled in this study, including 72 patients (73.5%) with non-Hodgkins lymphomas (NHL) and 26 patients (26.5%) with Hodgkin’s lymphoma (HL). The increased serum ferritin level (more than 350 ng/ml) was found in 53 (54.2%) patients with malignant lymphomas at the onset of disease and its average concentration was 587,62±131,6 ng/ml (8.3 times higher values of control group, p<0.001).Also the positive statistical correlationsbetween increased ferritin level and increased level of LDH (r=0.47, p<0.001, n=98) and C-reactive protein (r=0.41, p<0.001, n=98) as well as the presence of B-symptomswere found. The median OS was significantly shorter in the group of patients with increased ferritin level (more than 350 ng/ml) at the onset of disease in comparison with group of patients with normal ferritin level, where the median OS was not reach during the observation period. Patients with increased ferritin level before starting chemotherapy also showed worse results of overall survival and increased mortality risk (OR 8.122; 95% CI, 1.764-37.396;р<0.05) compare with a group of patients with ferritin level ˂350 hg/ml at the onset of disease. Conclusion:These results make it possible to include lymphomas’s patients with increased ferritin level at the onset of disease in the group with poor prognosis and lower OS, while the increased ferritin level in patients without previous blood transfusions should be considered as a significant prognostic factor.


Hemoglobin ◽  
2021 ◽  
Vol 45 (1) ◽  
pp. 69-73
Author(s):  
Salah H. AL-Zuhairy ◽  
Mohammed A. Darweesh ◽  
Mohammed A-M. Othman

2015 ◽  
Vol 107 (1) ◽  
pp. 187-193 ◽  
Author(s):  
Sung Keun Park ◽  
Won Joon Choi ◽  
Chang-Mo Oh ◽  
Min-Gi Kim ◽  
Woo Taek Ham ◽  
...  

2020 ◽  
Vol 19 (3) ◽  
pp. 158-163
Author(s):  
E. E. Nazarova ◽  
D. A. Kupriyanov ◽  
G. A. Novichkova ◽  
G. V. Tereshchenko

The assessment of iron accumulation in the body is important for the diagnosis of iron overload syndrome or planning and monitoring of the chelation therapy. Excessive iron accumulation in the organs leads to their toxic damage and dysfunction. Until recently iron estimation was performed either directly by liver iron concentration and/or indirectly by measuring of serum ferritin level. However, noninvasive iron assessment by Magnetic resonance imaging (MRI) is more accurate method unlike liver biopsy or serum ferritin level test. In this article, we demonstrate the outlines of non-invasive diagnostics of iron accumulation by MRI and its specifications.


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