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Showing 93 results for Thalassemia

S. Rahgozar , A.r. Moafi , F. Yavari , H. Hourfar ,
Volume 1, Issue 2 (1-2005)
Abstract

  Abstract

  

 Background and Objectives

  Mismatched red blood cell phenotypes between donors and recipients in multiple blood transfusions can result in the development of alloimmunization in recipients. We studied in this research the effect of alloantibodies on the increase of need of blood transfusion in major thalassemiacs.

  

  Materials and Methods 

  This is a descriptive study in which 2 groups of major thalassemiacs with more and less than 20 days of blood transfusion intervals (27 patients vs. 25) were evaluated for the presence and frequency of alloantibodies and related factors. We used t-test and t-student tables for evaluating the results.

 

 Results

  55% of patients in the first group had developed alloantibodies and their annual transfused blood volume was more than those who were not immunized (p<0.005). Male gender and initial blood transfusion in children under 3 years old were related to the absence of alloantibodies. 100% of patients in the second group were immunized, and those who received higher amounts of blood units annually (493 ml/kg and 508 ml/kg) were patients with more than two types of alloantibodies. Alloimmunization involved K (27.5%), N (12.5%),
CW, s, Fyb (5%), C, S, E, e and M (2.5%) antigens. 100% of antibodies were of warm immunoglobulin type, and 16% both warm and cold. 17.3% of thalassemiacs were splenectomized and their need for transfused blood was less than unsplenectomized patients (p<0.005). In most cases, annual blood transfusion in both groups was estimated to be much more than what was expected.

  

 Conclusions

  We conclude that red blood cell matching, at least for Kell and Rh systems, is necessary to prevent alloimmunization in thalassemiacs. Hypersplenism and low quality of blood, that can increase the need for transfused blood, should be taken into consideration.

  

  

 Key words: Alloantibody, Major thalassemia, Blood transfusion


M. Aghaeipour , F.a. Tarabadi , M. Shaeigan , G.r. Babaee ,
Volume 1, Issue 2 (1-2005)
Abstract

  Abstract

  

 Background and Objectives

 Cytomegalovirus (CMV)infection has been recognized as a complication of blood transfusion. Transfusion-related CMV infection produces dramatic problems in immunocompromised patients including organ transplantation recipients, AIDS patients under immunosuppressive therapy, thalassemia major patients, and premature neonates. Regarding the importance of this infection in multitransfused patients and differences in prevalence of transfusion – related CMV infection in various reports, especially in thalassemia major patients, we decided to detect and compare the prevalence of CMV antibodies in thalassemia patients and blood donors.

 

  Materials and Methods 

 In this study we detected anti-CMV antibodies (IgG,IgM) by Elisa technique. We tested these antibodies in 55 thalassemia major patients (45 non-splenectomized and 10 splenectomized) and 1040 healthy donors.

 

 Results

 Our results showed that anti-CMV IgG antibody was positive in 89.6% of control group and 100% of thalassamic group, thus indicating of no significant difference in these two groups. Anti-CMV IgM antibody was positive in 0.04% of control group and in 9.1% of thalassemic group showing a significant difference. The prevalence of this antibody was respectively 30% and 4.5% in splenectomized and non-splenectomized groups of patients.

  

 Conclusions

 Absence of significant difference of anti-CMV IgM antibody in patient and control group reflected high frequency of this infection in our population and also the importance of the use of leukocyte free products for high risk blood recipients. Moreover, the significant difference in anti-CMV IgM antibody in splenectomized and non-splenectomized patient groups is related to the role of spleen in clearance of infectious agents and production of IgM antibody in this organ

  

  

 Key words: Cytomegalovirus (CMV), Thalassemia major, Elisa, Blood donor, Anti-CMV antibodies 


J. Abdy , M. Shaiegan ,
Volume 2, Issue 3 (3-2005)
Abstract

  Abstract

  

 Background and Objectives

  The immunity system of thalassemics cause of different reasons faces dysfunction. So they are susceptible to recurrent infections. One of these reasons pertains to deficiency in phagocytosis, chemotaxis, and bacterial killing ability neutrophils. As there are several reports about decline in neutrophilic functions in such patients, we aimed at studying phagocytosis and candida killing in major thalassemics.

  

  Materials and Methods 

  In this study, we analyzed blood samples drawn randomly from 30 patients with thalassemia major (16 splenectomized), and 30 healthy subjects as control group without any familial background of the disease. We evaluated phagocytosis and candida killing in these groups.

 

 Results

  Our findings showed that there is no relationship between sex and age with neutrophil function in the patients. There is a significant difference in phagocytosis (p<0.1) and killing ability between the patient group and control group (p<0.0001).

  

 Conclusions

  Since phagocytosis and candida killing are of lowest activity in patients than in the control group, there may be relative deficiency in these patients’ neutrophils function.

  

  

Key words : Thalassemia major, Neutrophil, Phagocytosis
A.r. Moafi , S. Rahgozar , A.a. Pourfathollah , M.m. Hariri ,
Volume 2, Issue 3 (3-2005)
Abstract

  Abstract

  

 Background and Objectives

  Blood group antigens include more than 260 antigens on the erythrocyte cell membrane, forming 24 different blood group systems. These can be categorized in 11 major systems which account for 172 of the antigens. Incompatibility of some of these antigens, especially in multiple transfused patients, results in hemolytic reactions. Although intermediate thalassemics need fewer blood transfusions than major ones, alloimmunization is more frequent in the former. This can be due to the different frequenct rates of blood group phenotypes in intermediate thalassemics as compared with healthy people.

  

  Materials and Methods 

  Red blood cell antigen typing was done in 39 intermediate thalassemics and 150 healthy blood donors for Rh, Kidd, MNSs, Lutheran and Duffy blood systems. K and M antigens had different frequency rates in both groups (p<0.01, p<0.0005) showing higher frequency in patients.

 

 Results

 Complementary studies are recommended to be performed in order to evaluate high-risk alloimmunization in intermediate thalassemias. Assessment of the frequency of other red blood cell antigens, antibody screening, and identification of alloantibodies in immunized intermediate thalassemics can be helpful in this regard.

 

 Conclusions

 Kantigen is very immunogenic however its prevalence rafe is high among intermediate thalassaemics, but this contradiction can not be posed as the increasing cause of alloimmune reactions in such patients. Supplemental studies including screening and detection of post-transfusion created antibody types not only help in selecting the more appropriate blood type for thalassaemics but also help in justifying increasing alloimmune reactions in thalassaemics.

  

  

Key words : Blood group antigens, Thalassemia , Immunization
S.e. Torabi , K. Abed Ashtiani , R. Dehkhoda , A.n. Moghadam , R. Sorkhabi , M.k. Bahram , J. Babaei ,
Volume 2, Issue 4 (6-2005)
Abstract

  Abstract

  

 Background and Objectives

  Patients with major b -thalassemia have been exposed to a wide range of blood-borne viruses among which hepatitis B and C viruses have a considerable importance. In this study, seroprevalence and risk factor of HBV, HCV and HIV infections among thalassemic patients of East Azarbaijan were evaluated.

 

  Materials and Methods

  Eighty four thalassemic patients who were regularly transfused at Children Hospital (Tabriz) enrolled in this observational study. Some data were collected through questionnaires and patients’ medical history then, patients were evaluated for the presence of HCVAb, HBsAg, HIVAb, HBsAb, HBcAb and ferritin by ELISA moreover, HCV positive sera were confirmed by Recombinant Immunoblot Assay (RIBA-3.0).

 

 Results

 None of the eighty-four thalassemic patients were HIV and HBV positive however, 6 (7.1%) were HCV positive. Distribution of sex and the mean ferritin did not differ from HCV-positive to HCV-negative patients, but the age average was significantly higher in HCV-positive patients (p<0.001). Moreover, HCV-positive patients had a significantly longer history of transfusion compared with HCV-negative patients (p<0.001).

 The prevalence of HCV in those patients who had received their first blood transfusion before the implementation of compulsory testing for HCV in 1995 was significantly lower. In other words, no case became infected after the initiation of donor screening test for HCV.

 

 Conclusions

  The prevalence of HCV in thalassemic patients of East Azarbaijan was lower compared to other provinces of Iran and all positive cases in this study went back to the time period preceeding 1995 that is before the implementation of compulsory HCV screening tests.

  

  

 Key words: Major thalassemia , Hepatitis B, Hepatitis C


M.r. Pourkarim , G.r. Khamisipour , G.r. Hajiani , R. Tahmasebi , N. Ardeshirdavani ,
Volume 2, Issue 4 (6-2005)
Abstract

Abstract

 

Background and Objectives

HTLV is a member of Retroviridae family whose infection can lead to leukemia. Since in most regions of Iran (like Bushehr) donated blood units are not tested for HTLV infection, multiple transfusion patients are at high risk. In this study, we evaluated serological prevalence of the infection among Busherian patients.

 

Materials and Methods

This was a descriptive study in which samples of all Busherian multi-transfused patients were taken based on dispersion of thalassemic and hemodialysis hospital wards. Blood samples for detection of HTLV I/II infection markers were tested by ELISA method and for  confirmation of positive ELISA results western blot technique was used. Data were analyzed by using SPSS software and Chi-square.

 

Results

Out of 642 Busherian multi-transfused patients including 455 (70.78%) thalassemic, 86(13.3%) hemophilic and 101 (15.7%) hemodialysis patients, 41 all thalassemic were ELISA positive out of whom 22(53.7%) were male and 19(46.3%) female. Infection in 14 cases was confirmed by western blot technique (p=0.77). Statistical analysis did not show any significant correlation between western blot results and gender (p=0.56). Neither was there any significant difference between western blot test results across different age ranges (p=0.67). The significant point in this research was the detection of a thalassemic patient with dual infection of HTLV type I and II.

 

Conclusions

Detection of HTLV infection among multi-transfused population indicated that such an infection may exist among blood donors of Bushehr.

 

 

Key words:  Transfusion HTLV I/II, Thalassemia, Hemophilia


A. Azarkeivan , A. Sadri , Sh. Ansari Damavandi ,
Volume 2, Issue 4 (6-2005)
Abstract

  Abstract

  

 Background and Objectives

  Thalassemia is the most prevalent hereditary anemia worldwide and in our country. Lifelong blood transfusion is currently the treatment protocol of this disease, but it has some side effects one of them is alloimmunization. Alloantibodies produce antigens against RBCs which patients do not have. The antigens are transmitted via chronic blood transfusion gradually. One of these RBC antigens is antigen I, and the antibody against it is IgM class and cold antibody. Cold agglutinins are capable of agglutinating RBCs in low temperature and may result in hemolytic anemia which is known as cold agglutinin disease (CDA). In Iran, data regarding incidence of CAD are not available, but incidence of CAD in USA is approximately 1:300000.

 

  Case

  An 18-year-old man with b -thalassemia major referred to the hematology lab for periodic checking of blood smear. He had clumps of RBC and RBC indices were abnormal. In repeated examination, he was known to be asymptomatic for purpose of cold agglutinins.

 

 Conclusions

  We do not have any records or information about cold agglutinins in thalassemic patients in Iran. This case was detected only through periodic laboratory tests without any special complications on the part of the patient .

  

  

 Key words: b -thalassemia major, Hemolysis, Cold agglutinins, RBC clumps


A. Gharehbaghian , S. Tavakoli , S. Amini Kafiabad , A.h. Zarnani ,
Volume 2, Issue 5 (9-2005)
Abstract

  Abstract

 Background and Objectives

  The prevalence of GBV-C and HGV in blood donor populations in developd countries based on HGV RNA detection and anti-E2 screening ranges from 1 to 5 and 3 to 14% respectively. The aim of this study was to investigate seroepidemiologic hepatitis G virus (HGV) in blood donors, haemodialysis patients, haemophiliacs, and β thalassemics with a history of liver disease by Elisa tecnique.

 

  Materials and Methods

  In this descriptive study, blood samples of 330 volunteer blood donors, 44 haemodialysis patients, 16 haemophiliacs, and 40 β major thalassemics with a history of liver disease were studied by Elisa technique for their seroepidemiologic status of hepatitis G virus and their past record of HGV infection. For data analysis, Chi-square, Fisher exact test, and SPSS version 11.5 were used.

  

 Results

  This study showed that out of 330 healthy blood donors 14(4.2%), out of 44 haemodialysis patients 10(22.7%), out of 16 haemophiliacs 5 (30.3%) and out of 40 β thalassemics 10 (25%) were positive for HGV-anti-E2. These data are significant evidence for HGV to be considered as a transfusion-transmitted infection. The prevalence of anti-HGV and anti-HCV (co-infection) was found to involve 10 (30.3%) of haemodialysis patients, 4 (28.6%) of haemophiliacs and 9 (23.7%) of β thalassemics. It was also found that 1 (8.3%) of haemodialysis patients, 1 (33.3%) of haemophiliacs, and 1 (50%) of β thalassemics were infected with anti-HGV and HBsAg co-infection.

 

 Conclusions

  The prevalence of HGV was high in multitransfused individuals including haemodialysis patients, haemophiliacs, and thalassaemics. Therefore, HGV was a transfusion-transmittable agent. Co-infection of anti-HGV with HCV was observed in viruses. It is recommended that further studies focus on evaluating sexual and vertical transmission routes so as to cast light on relatively high rate of HGV in donor population.

  

  

 Key words: HGV, Blood donors, Haemodialysis, Haemophilia, Beta thalassemia


Ghafouri M., Mostaan Sefat L., Sharifi Sh., Hosseini Gohari L., Attarchi Z.,
Volume 2, Issue 7 (1-2006)
Abstract

  Abstract

 Background and Objectives

  Distinguishing two common hypochromic microcytic anemias (i.e. iron deficiency anemia and minor thalassemia) needs expensive laboratory tests not readily available everywhere. A number of indices derived from original cell counter parameters were tried to screen patients these indices are compared in this study.

  

 Materials and Methods

  Four indices of England, Mentzer, Shine and MDHL were calculated for 110 cases of known thalassemia minors and 61 iron deficients. The best cut-off point for each index was selected by receiver operator characteristic (ROC) analysis reaching the highest sensitivity to separate thalassemics from iron deficients. Sensitivity and specificity of indices were compared.

 

 Results

  Mentzer’s index(sensitivity:90.9, specificity:80.3) had the best results.

 

 Conclusions

  It seems Mentzer’s index can be used widely to screen hypochromic microcytic anemic patients. Tests like hemoglobin electrophoresis, genetic studies, serum iron, TIBC and ferritin measurement should be reserved for selected patients in refferal centers. In this way we reduce public health costs significantly.

  

 Key words : Differential diagnosis, Minor thalassemia, Iron deficiency


Dr. H. Javadzadeh Shahshahani, M. Atar, Dr. M.t. Yavari, Dr. Sh. Savabieh,
Volume 2, Issue 7 (1-2006)
Abstract

  Abstract

 Background and Objectives

 As hemophilia and thalassemia patients are in regular need of blood or blood products, they are exposed to blood units of hundreds or even thousands of blood donors therefore, they are at a high risk of acquiring blood transmissible infections. Different prevalent rates of hepatitis B, C and HIV infection in hemophilia and thalassemia populations have been reported in various regions of Iran and the world. The present study was conducted to determine the prevalence of hepatitis B, C and HIV infection in hemophilia and thalassemia population of Yazd province of Iran.

 

 Materials and Methods

 In this descriptive study, 85 thalassemia major and 74 hemophilia patients were included. The necessary information was gathered from the patients, blood samples were tested for serum markers of anti-HIV, anti-HCV and HBsAg by the ELISA method, and positive cases were confirmed by Western blot, RIBA and neutralization methods. The data were analyzed by Chi square and Fisher exact tests.

  

 Results

 None of the thalassemia patients was positive for HIV Ab or HBsAg, while 9.4% were anti-HCV positive. In hemophilia patients, the prevalence of HIV Ab and HBsAg was 1.4%, while that of anti-HCV was 48.6%. The prevalence of anti-HCV in those patients who had received blood or blood products after implementation of the screening program in the country was significantly lower (P=0.02, P<0.000). Overall, the prevalence of hepatitis C as compared to hepatitis B and HIV was higher in both the hemophilia and thalassemia patients.

 

 Conclusions

 Hepatitis C is the main problem of these patients, especially hemophiliacs. The screening program in the country has led to a significant reduction in the incidence of hepatitis C. In order to reduce the risks of transmissible infections by blood and blood products in the future, it is proposed tests that are more sensitive be used, especially for reduction of the window period of hepatitis C. For hemophiliacs, virally inactivated factor concentrates should be used in case such concentrates are not adequately available , donor retested quarantined plasma should be used.

 


Raeissi N, Habibian R, Zandian K,
Volume 3, Issue 3 (10-2006)
Abstract

  Abstract

 Background and Objectives

 Hepatitis B virus (HBV) is one of the main causes of hepatitis, cirrhosis and hepatocellular carcinoma, and vaccination by anti-HBs is the only reliable method for its prevention. Since blood transfusion is one of the most important transmission routes of this virus, blood recipients may always be at its exposure. Considering the importance of vaccination in multitransfused patients, we decided to evaluate HBsAb level in them.

 

 Materials and Methods

 In this cross sectional study with simple sampling, 83 patients with major thalassemia, 5 with hemophilia, and 5 with fanconi anemia, having been referred to Hajar Hospital of Shahrekord to receive blood and blood products in the year 2003 were evaluated for their level of HBsAb. The level of HBsAb was measured with Diasorin kit (sensitivity=95%, specificity=98%). Data were statistically analyzed with chi-square test.

 

 Results

 Thirty patients (28.5%) were nonimmune, 11 (12.5%) were partially immune, and 52 (59%) were immune. Eighty eight patients had previous history of hepatitis B vaccination. Out of those who had received the last vaccination within the last 5 years, 82% were immune (p<0.05) and out of those who had it earlier, only 35.7% were immune.

 

 Conclusions

 Besed on the results of this study, determination of immune status in multitransfused patients and their immunization are important and revaccination after a 5-year lapse in patients is recommended.

  

 Key words : Hemophilia, Thalassemia, Vaccination, Transfusion 


Kiani A, Abdi J, Shirkhani Y, Kashi M,
Volume 3, Issue 3 (10-2006)
Abstract

  Abstract

 Background and Objectives

  Thalassemia major patients should continuously receive blood to survive. Permanent blood injection will expose patients' immune system to a broad spectrum of new antigens located on the surface of injected RBCs. Continuous exposure to foreign antigens may provoke antibody production against them in the patients who lack those Ags. This phenomenon is termed alloimmunization and can cause problems for preparation of compatible blood for transfusion.

 

 Materials and Methods

  65 patients were studied in this research. At first, questionnaires eliciting information about age, race, date of blood injection, splenectomy, presence of any underlying diseases, and any certain drugs(in patients' records) were filled out by all patients. For alloantibody screening, patients' serum samples were tested by the panels prepared in IBTO. Phenotypes of RBCs for Ags of ABH and Rh(D, C, E, c, e) were also determined by relevant antisera.

 


 Results

  Out of 65 patients, only one case (1.53%) had been allommunized. The age range of males and females were 13 ± 6 and 13 ± 5 respectively. All of the patients were of Lurish race. The first blood transfusion in all of them occurred at the age below 3. Seventeen subjects were splenectomized.

 

 Conclusions

  The reasons for low prevalence of alloimmunization against RBCs in thalassemia major patients may pertain to similarity of patients and donors by descent, blood transfusion before the age 3, and splenectomy in a few patients. Identification of alloantibodies by using the panel cells was the best method to provide patients with antigen negative and compatible blood for transfusion.

  

 Key words : Alloimmunization, Panel, Thalassemia major


B. Tabarsi, A. Marbaghi, M. Safavi, M. Afkhami,
Volume 4, Issue 1 (3-2007)
Abstract

  Abstract

 Background and Objectives

 Thalassemia is one of the important chronic hereditary diseases. There are nearly 20000 thalassemia major patients in Iran. Multiple blood transfusion and aggregation of iron load cause numerous complications which can be reduced by regular treatments. This study was conducted to determine and compare the problems of thalassemia major clients in regular and irregular follow ups of therapeutic principles.

 

 Materials and Methods

  This study was performed as a cross-sectional study. The sample consisted of all thalassemia major clients over 40 years of age having referred to the center of adult thalassemia in Tehran. 224 clients were divided into regular and irregular groups. A questionnaire was used for data collection.

 

 Results

  Based on difference between the averages of regular and irregular groups the average rate of psychological problems was 22.7%, sociological problems 18.7%, and economic problems 17.6%. Based on average differences between the two groups, important psychological problems include fatigue due to long periods of treatment (1.5), future hopelessness (1.4), and lack of self confidence (1.2) sociological problems include community unawareness of the disease (1.3), difficult access to various specialists (1.1), and lack of ongoing training classes (1.0) finally economic difficulties include access to necessary drugs and equipment (0.93), quality of free equipment (0.92), and family support (0.8). These are the prominent problems addressed in irregular treatment follow ups.

 

 Conclusions

  Results have revealed that psychological problems had a more distinct effect relative to social and economic problems on the therapeutic irregular follow ups.

  

 Key words : Thalassemia major, Patients, Iran. 


B. Keikhaei, F. Rahim, Kh. Zandian, M. Pedram,
Volume 4, Issue 2 (8-2007)
Abstract

  Abstract

 Background and Objectives

 Iron deficiency anemia (IDA) and ß thalassemia trait (TT) are the most common forms of microcytic anemia. Some discrimination indices calculated from red blood cell count and red blood cell indices are defined and used for rapid discrimination between TT and IDA. Youden's index is the most reliable method to measure the validity of a particular technique, because it takes into account both sensitivity and specificity.

 

 Materials and Methods

 We calculated 10 discrimination indices (Mentzer Index, England and Fraser Index, Srivastava Index, Green and King Index, Shine and Lal Index, red blood cell (RBC) count, red blood cell distribution width, red blood cell distribution width index (RDWI), Mean Density of Hemoglobin per Liter of blood (MDHL) and Mean Cell Hemoglobin Density (MCHD)) in 170 patients with IDA and in 153 patients with b TT (βTT). We divided the patients into two different age ranges of 1 to 10 and 10 to 57 years.

 

 Results

 We determined the number of correctly identified patients by using each discrimination index. None of the indices showed sensitivity and specificity of 100% in the latter group it was just Shine and Lal Index (S and L) that showed a sensitivity close to 90% and specificity of 100% in the former group. The accuracy order of these discrimination indicies from higher to lower for the former was Shine and lal > RBC Count > Srivastava > Mentzer > England and Fraser > Green and King > RDWI > RDW and in the latter RDWI > RBC count > Mentzer > England and Fraser > Srivastava > RDW > Shine and Lal > Green and King. Youden's index for Shine and lal and RBC Count, and for RDWI and RBC Count has the highest diagnostic value in the former and latter groups respectively. Mean Cell Hemoglobin Density (MCHD) and Mean Density of Hemoglobin per Liter (MDHL) did not show any diagnostic value.

 

 Conclusions

 None of the indices was completely sensitive and specific in differentiating between b TT and IDA. MCHD mean and median were very close to normal values for both IDA and b TT patients, but in case of MHDL we have found mean and median values being significantly higher than normal values in b TT and lower in IDA patients. In our study, Youden's index of RBC count and RDWI were the highest ones and they were the most reliable discrimination indices in differentiating b TT from IDA in the latter group while for patients in the former RBC and S & L were the most reliable discrimination indices.

  

 Key words : b thalassemia, Iron deficiency anemia, Indices 

 


M.a. Ehsani, E. Shahgholi, K. Sotoudeh,
Volume 4, Issue 2 (8-2007)
Abstract

  Abstract

 Background and Objectives

  While public focus is on the risks of transfusion transmitted infections, transfusion errors contribute significantly to adverse reactions.

 

  Case

  In this study we describe two cases of mistransfusion two thalassemic siblings were admitted for monthly transfusion: a 19 year old boy and his sister. The donor whose blood donation was cross matched for the sister had a name similar to the brother so it was wrongly administered to him that led to the hemolytic reaction. Another 2.5 year old boy with O+ blood group received A+ blood because of the staff error in recording the patient blood group and neglecting the crossmatch.

 

  Conclusions

  The most prevalant complications of blood transfusion are due to human error. In order to decrease errors, it is recommended to double check the blood order form and the information on the blood bag.

 

 Key words: Transfusion, Thalassemia, Complications, Human error

 


S. Alavi, M.t. Arzanian, M. Ahmadinejad, G. Meshkat Razavi, N. Valaee, M.r. Tabatabaee, M. Karbasizadeh,
Volume 4, Issue 3 (9-2007)
Abstract

  Abstract

 Background and Objectives

 Thrombotic events are among the most important complications in patients with beta-thalassemia major. Alterations in natural anticoagulation system have a major contribution to the occurrence of thrombotic events in these patients. This study was performed to evaluate the protein C, protein S and Antithrombin III activity in patients with beta-thalassemia major having referred to Mofid Children Hospital in 1382.

 

 Materials and Methods

 Thalassemic children older than 2 years of age, with the interval of more than 25 days since last transfusion, and without any evidence of symptomatic liver or heart disease were enrolled in the study. Plasma activity of protein C and protein S was assessed by PTT based tests and the activity of antithrombin III was determined by chromogenic method. The correlation between decreased levels of anticoagulants and other parameters such as splenectomy, liver transaminases and serum ferritin level was analyzed by spss software.T-test and Mann whitney U-test were used to compare the mean values of variables.

 

 Results

 20.3% of patients showed a decrease in protein C and 15.3% in protein S. 12 patients had been splenectomized. Mean protein C activity in splenectomized and non-splenectomized patients were 63.4 ± 17.9% and 74.5± 15.1% , respectively (p<0.04).

 

 Conclusions

 There was a decrease in proteins C and S activity in thalassemia major patients. Splenectomy might cause reduction in protein C level. Analytic studies are recommended to evaluate the activity of anticoagulant system in thalassemic patients. In addition, assessment of this system is suggested prior and after splenectomy .

 

 Key words : Thalassemia major, Thrombosis, Anticoagulant, Splenectomy

 


M.a. Ehsani, K. Sotoudeh, E. Shahgholi, M.s. Rahiminejad, F. Seighali, A. Aslani,
Volume 4, Issue 3 (9-2007)
Abstract

  Abstract

 Background and Objectives

 Iron deficiency anemia (IDA) and beta thalassemia minor (BTM) are the most common causes of hypochromic microcytic anemia. Many indices have been defined for rapid differentiation of these diseases via red blood cell indices. The objectives of this study were to introduce a new index, evaluate its validity, and compare it with other indices.

 

 Materials and Methods

 In this descriptive observational study, new patients with hypochromic microcytic anemia referred to hematology clinic were studied. 130 patients with IDA and 154 with BTM with the mean age of 24.2 years were assessed. Inclusion criteria were anemia [Hemoglobin (Hb) 2 were measured. BTM and IDA diagnoses were confirmed with HbA2> 3.5% and ferritin < 12 ng/ml or anemia responding to iron respectively. All patients were assessed according to red blood cell (RBC), Mentzer index, England index, Srivastava index and red cell distribution width, and our new index: [MCV-(10 . RBC)]. Sensitivity, specificity, as well as Youden's index have been calculated for all indices.

 

 Results

 Only Mentzer index and the newly introduced index had the sensitivity and specificity of over 90%. Mentzer Index and the newly introduced index had the highest Youden's index.

 

 Conclusions

 Compared to other well-known indices, the newly introduced index is straightforward and has a good sensitivity and specificity to discriminate IDA and BTM. Its calculation is easy and does not need calculator.

 

 Key words : Iron deficiency anemia, Beta thalassemia, Erythrocyte indices

 


A. Azarkeivan, H. Najmabadi, F.s. Esteghamat, H. Imanian, S. Ebrahimkhani,
Volume 4, Issue 3 (9-2007)
Abstract

  Abstract

 Background and Objectives

  Thalassemia is a congenital anemia which is endemic in our region. In our country the national prevention program is followed for the couples who want to get married to be screened via CBC test then, according to the results, complementry tests are conducted. Through this program the carriers will be diagnosed and new cases might be avoided by prenatal diagnosis.

 

  Case

  We had a 2.5 year old patient with anemia who came to our thalassemia clinic for exact diagnosis. In her history, her mother had minor beta thalassemia and her father had Hb D or G in Hb electrophoresis pattern. Their hematology consultation before showed that their infant will not have any chance of being affected with major thalassemia. CBC, Hb electrophoresis ( Cellulose Acetate pH:8.4) and molecular diagnosis of beta genes were performed for parents and the child. In hematological study, the patient had low Hb level, high reticoulocyte count (10%) and severe morphologic changes in smear. Hb electrophoresis pattern showed 15.9% HBA, 79 % HbF and 4.1% HbD or G. Genetic study showed IVS II-I/N for mother and Hb Lepore/N, Hb D negative for father. The patient was Hp Lepore/IVS II-I which is the combined form of Hb Lepore and b thalassemia mutation. The diagnosis indicated of hetrozygote b thalassemia and Lepore hemoglobin which is the same as intermedia thalassemia phenotype.

 

  Conclusions

  Hemoglobin Lepore is a variant of thalassemia syndrome with the crossing over of chromosome 11. In Hb electrophoresis, hemoglobin Lepore band is overlapped with Hb D or G and can not be differentiated by these laboratory methods. In our country, in screening program for prevention of thalassemia, we have many cases with thalassemia minor. Since Hb Lepore electrophoresis band overlaps with Hb D or G, in Hb D or G patients who want to get married with other hemoglobinopathy patients, the possibility for Hb Lepore to go undetected should be considered.

 

 Key words: Hb Lepore, Thalassemia, Differential diagnosis

 


N. Cohan, M. Ramzi,
Volume 4, Issue 4 (2-2008)
Abstract

  Abstract

 Background and Objectives

 Beta thalassemia minor is a common form of microcytic anemia. Although it needs no treatment, prenatal screening of beta thalassemia followed by medical counseling for prevention of birth of children with beta thalassemia major is important before marriage. The specific criterion for beta thalassemia minor diagnosis is the elevated level of Hb-A2. Some discrimination indices helpful in detection of beta thalassemia minor were reported. The aim of this study was to define the sensitivity, specificity, and Youden's index for Kerman index I and II in screening of beta thalassemia minor.

 

 Materials and Methods

 A total of 82 patients with microcytic anemia (MCV< 80 fl) including 42 with beta thalassemia minor and 40 with iron deficiency anemia were selected. Differential values of Kerman index I and II were calculated then, the sensitivity, specificity and Youden's index were evaluated.

 

 Results

 In screening of beta thalassemia trait, sensitivity, specificity, and Youden's index were observed to be 93%, 87%, and 80% for Kerman index I respectively for Kerman index II these figures were calculated to be 76%, 90%, and 66% in order.

 

 Conclusions

 Although none of the discrimination indices showed complete sensitivity and specificity, but Kerman index I with higher sensitivity and specificity came out to be more valid in screening of beta thalassemia minor.

 

 Key words : Beta thalassemia, Sensitivity and specificity, Erythrocyte indices.


Sh. Khatami, S. Rouhi Dehboneh, S. Sadeghi, P. Saeidi, R. Mirzazadeh, P. Bayat, A. Amirkhani, A. Samavat, S. Zeinali, M.t. Akbari, H. Najmabadi,
Volume 4, Issue 4 (2-2008)
Abstract

  Abstract

 Background and Objectives

 Globin chain synthesis and DNA analysis are among complementary tests for thalassemia diagnosis. Nowadays, DNA analysis is the only definitive method for diagnosis of suspected carriers. Despite the complexity of this heterogenic disease which is attributed to mutations in gene regulation sites or unknown mutations, globin chain synthesis has maintained its significant role in identifying different kinds of thalassemia. As a result, besides the routine application of this method , we decided to determine the ranges of α/β ratio values in different kinds of thalassemia.

 

 Materials and Methods

 In this experimental study 214 cases were divided into the control (51 cases), minor β thalassemia (24 cases), mild α (α thal. 2) thalassemia (68 cases), severe α (α thal. 1) thalassemia (44 cases), Hemoglobin H disease (6 cases), silent β ( type II) thalassemia (14 cases), δβ thalassemia (5 cases), and αδβ thalassemia (2 cases) groups. CBC, hemoglobin electrophoresis using acetate cellulose paper in alkaline pH, hemoglobin A2 measurement by column chromatography, reticulocytes percentage, hemoglobin H, RBC morphology, and globin chain synthesis were performed on each group.

 

 Results

 Significant differences were observed in mean values of RBC, hemoglobin, hematocrite, MCV, MCH, MCHC, α/β ratio in α and β thalassemia cases as compared with the control group. High prevalence of α thalassemia was observed among suspected individuals (55.2% of different kinds of α thalassemia vs. 9.8% of different kinds of atypic β thalassemia) as compared with atypic β thalassemia.

 

 Conclusions

 The mean value of α/β ratio achieved in this study was similar to the others, but with a greater standard deviation. Because of this, there exists a wider range of α/β ratio. This width of range made overlaps in different and adjacent groups. Therefore, α/β ratio cannot be used by itself to firmly diagnose the type of thalassemia. As a result, for accurate diagnosis to be made, besides considering patient's ethnicity and clinical features, it is necessary to assess the results of CBC, hemoglobin electrophoresis pattern analysis, globin chain synthesis, familial tests, and DNA analysis.

 

 Key words : β Thalassemia, α thalassemia, Globin



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