Showing 8 results for Thalassemia
F Niknezhad, Kh Kalavi, M Mofidi, A Sarikhani,
Volume 1, Issue 1 (4-2007)
Abstract
Abstract Background & Objectives: Thalassemia syndromes isone of the inherited disorders in which one or more globulin chains are affected. On the basis of clinical symptoms, thalassemias are categorized as minor, intermediate, and major. Minor beta -thalassemia is a mild microcytic hypo chromic anemia in most cases asymptomatic and HbA2 is more than normal. Materials & Methods: This study carried out on 813 blood samples obtained from male high school students of Aliabad Katool, in the north of Iran. After detecting red blood cell indices of the subjects whose MCV was less than 80fl HbA study performed. Results: The MCV of 8.24% of the subjects was less than 80fl.34 of 67 had HbA2 of less than 3.5 %(normal) while the rest more than 3.5%(minor). Conclusions: Since about 50% of microcytic anemic patients were the carrier of beta-thalassemia and it is highly Prevalent in this region (4.06%), we recommend to investigate HbA2 in people with MCV less than 80 fl. Keyword: Thalassemia, Thalassemia minor, HbA2, Iran
S M Hedayat Mofidi, F Kouhsar, H S Hedayat Mofidi,
Volume 4, Issue 1 (4-2010)
Abstract
Abstract Background and objectives: Recurrent Abdominal Pain (RAP) syndrome is a common disorder, especially in children with beta thalassemia major. These patients are predisposed to heart diseases which are caused by hemochromatosis (Iron overload), resulting in sudden death. Because of the role of Helico bacter pylori in causing abdominal pain and peptic ulcer, and in increasing the risk or exacerbating of heart diseases, it can be important in beta thalassemia major. This study aimed at determining the frequency of anti- H.pylori class IgA in patients suffered from beta thalassemia major. Material and Methods: In this descriptive-analytic Study, 132 beta thalassemia major Patients and 135 healthy cases were matched by age, sex and ethnic as a control group. The sera were separated from clot in first Seroepidemiology and along with the filled questionnaires sent to Immunology-hematology Laboratory of paramedical school, in which the samples were tested by ELISA method with Diagnostic kit for anti-H.pylori IgA and IgG classes detection. We analyzed the Data by chi square and Independent T and Fisher tests. Results: the results show that the difference between the anti-H.pylori class IgA in case (22.7%) and control group (17.8%) is not significant (P=0.6), But it is significant between anti-H.pylori IgG class, 81.8% for cases and 54.8% for controls (P<0.001). Conclusion: Based on the results, the rate of anti-H.P IgA class in case is 1.131 times more than control group, which is not significant. On the other hand , the rate of anti-H.P IgG is 1.478 times more than control group which is significant. Key words: Thalassemia, Helicobacter pylori, Gorgan, Sero epidemiology.
Jafari M, Khalojini M, Falahati M, Ghadimipoor H R, Bigvand P, Tavakoli S,
Volume 5, Issue 1 (4-2011)
Abstract
Abstract Background and objectives: Candida albicans is a normal flora in skin and mucosa and is not pathogen in normal individuals.The main defence against yeasts ,particulary Candida, is phagocytes, but phagocytesis and opsonization are reduced in thalassemia . the aim of this study was the determination of the frequency of Candida albicans and its species in the oral cavity of patients with major thalassemia and normal individuals. Material and Methods: This descriptive –analytic study was Conducted on 52 patients with major thalassemia and 104 normal individuals in Hamadan,2009. The specimens from oral cavity of the patients and control group were taken by sterile swab and then the direct exam and culture of all specimens were done Results: The Candida species in patients with thalassemia are Candida albicans (32.7%), tropicalis (13.5%) glaberata(10.5%), guilliermondii(10.9%) and Krusei(10.9%). In normal individuals,the frequency is 17.3%for albicans, 3.8% For tropicalis ,1% for glaberata and 1%for kefyr.Fifty-nine percent of the subjects undergone splenctomy and 23% of those with heathy Spleen have Candida in their oral cavity, this differene is statistically meaningful. Conclusion:The results show that suffering from thalassemia and spleenectomy can increase the possibitity of oral infection by Candida species. Key words: Major thalassemia,Candida albicans,Oral Cavity.
S Royani, S Alijanpor, Z Shirbaghaei, R Khorasaninejad, Gh Roshandel, Aa Ayatollahi, Hr Joshaghani,
Volume 7, Issue 3 (10-2013)
Abstract
Abstract
Background and Objective: Of the most common hypochromic microcytic anemia are iron deficiency anemia and minor thalassemia, which are common in Iran and their differential diagnosis is extremely important. The level of 25-hydroxy vitamin D is the indication of vitamin D blood status. The aim of this study was to compare serum levels of vitamin D in people with minor thalassemia and iron deficiency anemia with healthy subjects in order to investigate the relationship between vitamin D deficiency and iron absorption.
Material and Methods: In this case-control study, 24 patients with minor thalassemia, 20 patients with iron deficiency anemia and 24 healthy individuals participated. Groups were matched for age and sex. Testing of Vitamin D level by ELISA, ferritin by quantitative luminescence method and HbA2 by column chromatography was carried out.
Results: The number of individuals with low level of vitamin D in iron deficiency group is 15 (75%), in minor thalassemia group is 8 (33/3%) and in the control group 11 (45.8%).
Conclusion: In this study, the highest percentage of vitamin D deficiency is observed in cases with iron deficiency anemia. Because of association between vitamin D and anemia, iron and vitamin D supplementation is recommended to enrich the diet.
Keywords: Anemia Minor Thalassemia Iron Deficiency Anemia Vitamin D
Fatemeh Asadi , Seyedeh Moloud Rasouli Ghahfarokhi , Forough Talebi ,
Volume 13, Issue 2 (3-2019)
Abstract
ABSTRACT
Background and Objectives:
Hemoglobinopathies are characterized by defects in the synthesis of globin chains of hemoglobin (Hb). The purpose of the present study was to evaluate mutations associated with
thalassemia and other
hemoglobinopathies in Masjed Soleiman County, Iran.
Methods: This descriptive study was carried out on 456 individuals suspected of having hemoglobinopathies who were referred to health centers of the Masjed Soleiman Country in 2015-2017. Blood samples were collected in EDTA tubes. Complete blood count test was performed and red blood cell indices were determined. Level of Hb variants was measured using capillary electrophoresis. Reverse dot-blot, gap-polymerase chain reaction and Sanger sequencing were carried out to detect mutations.
Results: We found that 17.7% of the subjects were heterozygous for β-thalassemia. Frequency of mutations 36
/37 (–T), IVS-II-1 (G
>A) and IVS-I-110 (G
>A)
in the β-globin gene was 26.7%, 22% and 16.27%, respectively. In addition,
9.5% of the subjects contained Hb S, Hb D and Hb C, while 1.1% of the subjects showed co-inheritance of an Hb variant and β-thalassemia. In subjects with α-thalassemia, the -α3.7 (57.1%), --
MED–(17.4%), -α
4.2 (3.1%) and -
α20.5 (1.5%) deletions were found as the most prevalent mutations.
Conclusion: In addition to the high prevalence of β-thalassemia and HBB gene mutations, we detected variants Hb S, Hb D, Hb C and co-inheritance of an Hb variants and β-thalassemia in individuals living in the Masjed Soleiman Country. We also identified four mutations in the α-globin gene. These results can be useful for genetic counseling in this population.
- : Hemoglobinopathies, β-Thalassemia, α-Thalassemia, mutation, HB variant.
Aradhana Harrison , Aswathy Prabha , Karishma Krishna , Vejay Viknesh Marudhadurai , Jahnavi Chikkegowda, Rajshree Choudhary ,
Volume 18, Issue 5 (9-2024)
Abstract
Background: β-thalassemia trait (BTT) can be screened by several discriminator indices (DIs) using complete blood counts (CBC). These DIs can help differentiate BTT from other causes of anaemia, thus reducing the financial burden of laboratory testing. At standard cut-off values, statistical analyses traditionally used to compare the diagnostic competence of these DIs give variable results. This study establishes new optimal cut-off values to improve the applicability of these DIs for BTT screening.
Methods: This was a retrospective study conducted on anaemic adults whose high-performance liquid chromatography (HPLC) and CBC results achieved over the past 6 months were reviewed. Based on HPLC reports, patients were categorised into BTT and non-BTT groups, with each group comprising 25 age- and sex-matched patients. Discriminator indices, including Mentzer’s Index (MI), Green and King Index (GKI), Sehgal Index (SI), Shine and Lal Index (SLI), Srivastava Index (SrI), and England and Fraser Index (EFI), were calculated for both groups. Statistical analysis was performed respective to standard cut-off values to establish new optimal cut-off values with the highest sensitivity and specificity.
Results: According to the results, SrI emerged as the best index, offering high sensitivity, specificity, Youden’s Index, accuracy, and odds ratio. On the other side, SLI and GKI were observed to be poor indices with low sensitivity and specificity. The new optimal cut-off values for the best performance of each DI for BTT screening were as follows: SrI ≤3.5, MI ≤11.4, GKI ≤59.7, SI ≤709.4, SLI ≤941.1, and EFI ≤1.91.
Conclusion: The performance of DIs at standard cut-off values was poor to screen BTT. New optimal cut-off values provided maximal sensitivity and specificity thereby enhancing their performance as screening parameters for BTT in regions with a high-prevalence of the condition. Further studies are warranted to substantiate the new cut-off values for BTT screening.
Parineeta Shelke , Preeti Doshi , Amit Nisal , Abdulrahaman Momin , Ravindra Nimbargi ,
Volume 19, Issue 2 (3-2025)
Abstract
Background: Hemoglobinopathies are a group of inherited disorders characterized by abnormal hemoglobin structure or synthesis, primarily classified into thalassemia syndromes and structural hemoglobin variants. Recognized as a global health priority, these disorders necessitate accurate diagnostic approaches. High-performance liquid chromatography (HPLC) has emerged as a reliable method for their detection. This study aimed to analyze the spectrum of hemoglobinopathies using HPLC in a population from Western Maharashtra, India.
Methods: A cross-sectional study was conducted at Bharati Vidyapeeth (DTU) Medical College, Pune, over three years. A total of 1,455 specimens from individuals of both genders were analyzed using the VARIANT™ II β-Thalassemia Short Program based on ion-exchange chromatography. Hematological parameters were assessed for all subjects, and the prevalence of hemoglobinopathies, along with their subtypes, was documented.
Results: The mean age of the study population was 26.91 ± 7.06 years, with a female predominance. The overall incidence of hemoglobinopathies was 8.78%, with β-thalassemia minor being the most prevalent, followed by β-thalassemia major, Hb S trait, and Hb S disease. Rare variants included two cases of Hb D Punjab heterozygosity and one case of Hb E heterozygosity. The Mentzer index exhibited a positive correlation with Hb F and mean corpuscular hemoglobin (MCH) and a negative correlation with hemoglobin levels, Hb A, and red cell distribution width (RDW-C).
Conclusion: The study revealed an 8.78% prevalence of hemoglobinopathies in the region, with β-thalassemia trait (Heterozygous) being the most common. Notably, co-inheritance of Hb E with β-thalassemia exacerbated anemia severity. These findings underscore the importance of HPLC-based screening for early diagnosis and management of hemoglobinopathies in high-risk populations.
Seyed Sadegh Baniaghil, Fardin Balochi, -- Ereste Kazak,
Volume 19, Issue 3 (7-2025)
Abstract
Background: One of the major complications in thalassemia patients is alloimmunization, which is antibody production by the patient against transfused RBCs (red blood cells). In the present study, the frequency of alloantibodies in thalassemia patients was investigated.
Materials and Methods: In this cross-sectional retrospective study, the study was performed on ninety nine multi-transfused thalassemia patients, antibody screening test was carried out using three cell-panel. Positive patients were followed up for antibody identification using 11-cell panel. The information was finally analyzed using SPSS software version 16.0.
Results: Out of ninety-nine cases, 53 were female (53.53 %) and 46 male (46.46 %). The patient’s age mean was 29.22±10.46 years with 2 to 61 years age range. Only 5.05% (N=5) had developed alloantibodies. The most common alloantibodies were Anti-D, Anti-E, Anti-c and Anti-K. No significant correlation was seen between the presence of alloantibody and age, sex, blood type and spleen condition.
Conclusion: Antibody production against RBC antigens is a common problem in multi-transfused thalassemia patients. Compatibility between antigens of Kell and Rh blood group systems in donors and recipients can be one of the useful ways to prevent alloimmunization of blood recipients and the production of unexpected antibodies against donor’s red blood cells.
