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Mehran Karimi - One of the best experts on this subject based on the ideXlab platform.
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contemporary approaches to treatment of Beta Thalassemia intermedia
Blood Reviews, 2012Co-Authors: Ali T. Taher, Khaled M. Musallam, Mehran Karimi, Maria Domenica CappelliniAbstract:Beta-Thalassemia intermedia (TI) is associated with a variety of serious clinical complications that require proactive and comprehensive management. These include skeletal deformities and osteopenia, compensatory extramedullary hematopoiesis and tumor formation, progressive splenomegaly, a hypercoagulable state resulting in thromboembolic events and pulmonary hypertension, and increased gastrointestinal iron absorption that often results in nontransfusional iron overload and liver damage. Although TI is generally considered a non-transfusion-dependent Thalassemia, transfusion therapy may be an important part of the comprehensive management of this disease. This review describes the current state of the art for medical management of TI, with particular focus on the roles of splenectomy, transfusion, and iron chelation therapy.
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Prevalence of hepatosplenomegaly in Beta Thalassemia minor subjects in Iran
European journal of radiology, 2007Co-Authors: Mehran Karimi, Mohammad Hadi Bagheri, Mehdi Tahmtan, Alireza Shakibafard, Murtaza RashidAbstract:Abstract Introduction Thalassemia is the most common hereditary blood disorder in the world. Iran is located on the thalassemic belt and there is a high prevalence of the hepatosplenomegaly in Beta Thalassemia minor patients which is reported to be very variable. The goal of this research was to study the frequency of these signs in the cases with Beta Thalassemia minor patients in Iran. Materials and methods Two hundred and fifty-nine cases that referred to center for pre-marriage tests were divided into two groups according to their MCV, MCH, and HbA2 (Beta Thalassemia minor cases and control groups). Liver and spleen sizes were determined by ultrasonographic method and the two groups were compared with each other. Results Average spleen volumes in case and control groups were 163.48 ± 133.97 and 126.29 ± 53.98 mm3, respectively. Average spleen lengths in case and control groups were 10.71 ± 1.52 and 10.60 ± 5.4 cm, respectively. Conclusion In the regions with high frequency of Beta Thalassemia, in case of finding large spleen size in the ultrasonography, a probable harmless differential diagnosis will be Beta Thalassemia minor that is not indicative of any serious disease. Volumetric measurement of spleen is more reliable for detection of splenomegaly in these patients.
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rbc alloimmunization in blood transfusion dependent Beta Thalassemia patients in southern iran
International Journal of Laboratory Hematology, 2007Co-Authors: Mehran Karimi, P Nikrooz, Sara Kashef, Nima Jamalian, Z DavatolhaghAbstract:Beta-Thalassemia is considered a severe, progressive anemia, which needs regular transfusions for life expectancy. One of the most important complications of regular blood transfusions may be alloimmunization, which increases the need for transfusion. This study was performed to investigate the production of red cell alloantibodies in Beta-Thalassemia patients in Shiraz, southern Iran. Blood sampling was performed among 711 Beta-Thalassemia patients in Dastgheib hospital in 2002-2004. Direct and indirect coombs tests were performed to check the auto and alloantibodies and a panel test was conducted to detect the type of alloantibodies. Auto and alloantibodies were observed among 1.7% and 5.3% of patients, respectively. The most common alloantibodies were Anti-kell (50%) > Anti-Rh (D) (15.8%) > Anti-Rh (E) (10.5%). All the patients who had developed alloantibody were in the age group of 6 years or more. So for decreasing the rate of alloantibody synthesis, we should crossmatched the packed cells for minor blood groups especially for kell and Rh(E) in addition to major blood groups from the start of transfusion.
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short stature in Beta Thalassemia minor subjects
Medical Science Monitor, 2004Co-Authors: Mehran Karimi, Hamdallah KaramifarAbstract:BACKGROUND Numerous disturbances in growth and development have been observed and demonstrated in homozygote Beta-Thalassemia patients. However, short stature in Thalassemia minor subjects, who have a minor defect in hemoglobin chain synthesis, has not yet been studied. MATERIAL/METHODS In this cross-sectional study, the heights of 100 Thalassemia minor subjects in the age group of 2-18 years and their parents were measured and analyzed. If the subject were in the 3-10 percentile range of height based on standardized sex and age curves, several follow-ups with complete history and physical examinations for a period of one year were preformed. If the healthy carrier's height was below the 3rd percentile, history, physical examination, and paraclinical examinations, including BUN, creatinine, electrolytes, serum alkaline phosphatase, thyroid function tests, growth hormone and cortisol levels, arterial blood gas, radiography of the left hand and wrist, etc, were also checked. One hundred healthy children were randomly chosen as a control group and matched for demographic characteristics with our healthy carrier subjects. RESULTS Mean +/- standard deviation for the age of our subjects was 6.62+/-3.63 years old. Twenty-seven (27%) of the patients had short stature. Except for hemoglobin (with a mean of 11.5 g/l), all other paraclinical data were normal. CONCLUSIONS We concluded that short stature was significantly more prevalent in the healthy Beta Thalassemia minor subjects than in the control group (p<0.001). Therefore, Thalassemia minor can cause short stature.
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response to hydroxyurea treatment in iranian transfusion dependent Beta Thalassemia patients
Haematologica, 2004Co-Authors: Majid Yavarian, Mehran Karimi, E Bakker, Cornelis L Harteveld, Piero C GiordanoAbstract:BACKGROUND AND OBJECTIVES: Hydroxyurea (HU) is known to increase gamma-globin chain expression in postnatal life. The efficacy of HU treatment in Thalassemia patients is still unclear. The aim of this study was to monitor treatment of a large cohort of patients with Beta-Thalassemia major in order to establish the response to HU and the associated elements. DESIGN AND METHODS: HU therapy was started in 133 patients diagnosed with transfusion-dependent Beta-Thalassemia in 1999. The molecular background of the disease, the polymorphisms of the promoter region of the genes, the haplotype of the Beta-globin gene cluster, the alpha-gene deletions and the HS2 polymorphism at the locus control region (LCR) of the Beta-globin gene cluster were studied. RESULTS: We were able to classify three categories of response: a good response (61%) in patients who shifted from monthly blood transfusion dependency to a stable transfusion-free condition at an average Hb level of more than 10 gm/dL; a moderate response (23%) in patients who remained transfusion dependent but at longer intervals (6 months or more), and non response in patients who, after one year of treatment, remained at the same level of transfusion dependency. The correlations with the molecular defects were found to be secondary to the presence of the (C-->T) at -158 of the Ggamma gene (XmnI polymorphism). The T allele, in linkage to the haplotype I (+\---|-) and to the internal Beta-globin gene framework 2, was the most significant modulating factor involved. INTERPRETATION AND CONCLUSIONS: The good response to HU treatment that a significant number of southwest Iranian patients with Beta-Thalassemia patients had seems to correlate with particular haplotypes. This indicates that HU treatment is a sensible option for transfusion-dependent Beta-Thalassemia patients with a favorable molecular background.
Piero C Giordano - One of the best experts on this subject based on the ideXlab platform.
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response to hydroxyurea treatment in iranian transfusion dependent Beta Thalassemia patients
Haematologica, 2004Co-Authors: Majid Yavarian, Mehran Karimi, E Bakker, Cornelis L Harteveld, Piero C GiordanoAbstract:BACKGROUND AND OBJECTIVES: Hydroxyurea (HU) is known to increase gamma-globin chain expression in postnatal life. The efficacy of HU treatment in Thalassemia patients is still unclear. The aim of this study was to monitor treatment of a large cohort of patients with Beta-Thalassemia major in order to establish the response to HU and the associated elements. DESIGN AND METHODS: HU therapy was started in 133 patients diagnosed with transfusion-dependent Beta-Thalassemia in 1999. The molecular background of the disease, the polymorphisms of the promoter region of the genes, the haplotype of the Beta-globin gene cluster, the alpha-gene deletions and the HS2 polymorphism at the locus control region (LCR) of the Beta-globin gene cluster were studied. RESULTS: We were able to classify three categories of response: a good response (61%) in patients who shifted from monthly blood transfusion dependency to a stable transfusion-free condition at an average Hb level of more than 10 gm/dL; a moderate response (23%) in patients who remained transfusion dependent but at longer intervals (6 months or more), and non response in patients who, after one year of treatment, remained at the same level of transfusion dependency. The correlations with the molecular defects were found to be secondary to the presence of the (C-->T) at -158 of the Ggamma gene (XmnI polymorphism). The T allele, in linkage to the haplotype I (+\---|-) and to the internal Beta-globin gene framework 2, was the most significant modulating factor involved. INTERPRETATION AND CONCLUSIONS: The good response to HU treatment that a significant number of southwest Iranian patients with Beta-Thalassemia patients had seems to correlate with particular haplotypes. This indicates that HU treatment is a sensible option for transfusion-dependent Beta-Thalassemia patients with a favorable molecular background.
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Beta Thalassemia intermedia from southern iran ivs ii 1 g a is the prevalent Thalassemia intermedia allele
Hemoglobin, 2002Co-Authors: Mehran Karimi, Maria Domenica Cappellini, Hooman Yarmohammadi, Shirin Farjadian, Sirus Zeinali, Zahra Moghaddam, Piero C GiordanoAbstract:The preliminary results of a pilot study are reported, intended as an initiation of a research plan, focused on the prevention of Beta-Thalassemia in Iran. The aims of this study are: (i) to improve the knowledge of the molecular background of Beta-Thalassemia intermedia in Southern Iran; (ii) to verify the role of the -158 (G)gamma (C-->T) (Xmn I) polymorphism as a modulating factor in Thalassemia intermedia; (iii) to test the validity of the multiplex and single mutation specific amplification refractory mutation system in analyzing the molecular defects causing Beta-Thalassemia in multiethnic populations; and (iv) to develop suitable strategies for the application of prevention protocols in Iran. To accomplish the task we have selected 87 Beta-Thalassemia intermedia patients and adapted the DNA methodology to detect the following 11 frequent mutations in Iran: codon 5 (-CT); frameshift codons (FSC) 8/9 (+G); codon 30 (G-->C); IVS-I-1 (G-->A); IVS-I-5 (G-->C); IVS-I-6 (T-->C); IVS-I-110 (G-->A); codons 36/37 (-T); codon 44 (-C); IVS-II-1 (G-->A); IVS-II-745 (C-->G). Because of the multiethnicity of the population we have also included the Indian IVS-I (25 bp deletion) and the Mediterranean IVS-I-130 (G-->C) and codon 39 (C-->T) mutations. Forty-eight patients were randomly studied for the Xmn I polymorphism together with 50 healthy volunteers as a control group. The molecular analysis conducted in Iran, identified only 31% of the alleles that were presumed to be thalassemic, revealing either a strategic or a technical insufficiency of the chosen method. However, the mutations with the highest prevalence in the country (IVS-II-1, IVS-I-110, IVS-I-1 and FSC 8/9) were found. As expected the IVS-II-1 defect, being the most frequent in south Iran, was present at the highest rate (24%). The Xmn I polymorphism was found in association with this prevalent mutation and was detected in the homozygous state in 87.5% of the patients homozygous for the IVS-II-1 (G-->A) mutation. The overall positivity for Xmn I was found in 40.6% of the thalassemic alleles vs. 14% in the non-thalassemic, confirming the hypothesis of an older event, antecedent to the IVS-II-1 mutation. In trying to assess a more suitable molecular detection method we intend to continue this study in collaboration with the European centers involved, applying more effective technologies and better defining the molecular spectrum of Beta-Thalassemia in the sub-populations. We also intend to verify the effect of alpha-Thalassemia in the genotype/phenotype correlation of Beta-Thalassemia intermedia.
Steven H. Kroft - One of the best experts on this subject based on the ideXlab platform.
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Iron deficiency anemia, Beta-Thalassemia minor, and anemia of chronic disease: a morphologic reappraisal.
American journal of clinical pathology, 2008Co-Authors: Alexandra M. Harrington, Patrick C. J. Ward, Steven H. KroftAbstract:We observed increased numbers of an infrequently referenced poikilocyte, the prekeratocyte, in iron deficiency anemia (IDA) compared with Beta-Thalassemia minor and anemia of chronic disease (ACD) and, therefore, chose to quantify these cells and other morphologic features in these anemias. Prekeratocytes were observed in 31 (78%) of 40 IDAs vs 11 (37%) of 30 Beta-Thalassemias (P = .001) and 5 (13%) of 40 ACDs (P < .001) and averaged 0.78 per 1,000 RBCs in IDA vs 0.21 in Beta-Thalassemia (P < .001) and 0.075 in ACD (P < .001). Pencil cells also were more commonly seen and more numerous in IDAs than in Beta-Thalassemia or ACD. Target cells were present in most IDAs and Thalassemia and in similar numbers. Basophilic stippling was seen in only 5 (17%) of the Beta-Thalassemias. Our results lend quantitative support to prekeratocytes and pencil cells as morphologic features favoring the diagnosis of IDA but fail to support the diagnostic usefulness of target cells and basophilic stippling in discriminating IDA and Beta-Thalassemia minor.
Helena Z. W. Grotto - One of the best experts on this subject based on the ideXlab platform.
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Measurement of reticulocyte and red blood cell indices in patients with iron deficiency anemia and Beta-Thalassemia minor.
Clinical chemistry and laboratory medicine, 2005Co-Authors: José F. A. Noronha, Helena Z. W. GrottoAbstract:New parameters correlated with the hemoglobin content in reticulocytes (RET-Y) and in red blood cells (RBC-Y) have been suggested as helpful in diagnosing iron deficiency anemia. We have studied RET-Y and RBC-Y indices in two groups of patients with microcytosis to verify if these parameters could be used to differentiate iron deficiency anemia from Beta-Thalassemia minor. Blood samples from 33 iron-deficient patients, 25 Beta-thalassemic minor patients and 50 normal individuals were analyzed on a Sysmex XE-2100 instrument. A significant difference was observed in reticulocyte counting and immature reticulocyte fraction between iron deficiency anemia and Beta-Thalassemia minor groups, but not in RBC-X and RET-Y parameters. Reticulocyte counting was higher in Beta-Thalassemia minor and the immature reticulocyte fraction was higher in severe iron deficiency anemia. The ratio RET-Y/mean cell volume was tested and was significantly different when Beta-Thalassemia minor was compared with mild and severe iron deficiency anemia, and showed better performance than the Mentzer ratio and the Green and King function. A great overlap of RET-Y and RBC-Y individual values was observed in both groups of microcytic anemias; we conclude that these new indices may be used with caution as indicative of iron deficiency, mainly in populations where Beta-Thalassemia minor is frequent.
Huizhu Zhong - One of the best experts on this subject based on the ideXlab platform.
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detection of alpha Thalassemia in Beta Thalassemia carriers and prevention of hb bart s hydrops fetalis through prenatal screening
Haematologica, 2006Co-Authors: Dongzhi Li, Can Liao, Jian Li, Yining Huang, Huizhu ZhongAbstract:The aim of this study was to detect alpha-Thalassemia in Beta-Thalassemia carriers during prenatal screening. During a 12-year prenatal screening program, a total of 158 couples (3.2%) were diagnosed to be the discordant alpha- and Beta-Thalassemia carriers. Of the 158 Beta-Thalassemia partners, seven (4.4%) were found to have co-inheritance of alpha0-Thalassemia, and three (1.9%) found to have co-inheritance of alpha(+)-Thalassemia. Three pregnancies affected with Hb Bart's hydrops fetalis were terminated in the 158 couples. The results showed that molecular analysis must be used for accurate diagnosis of double heterozygotes in couples presumed to be discordant for alpha- and Beta-Thalassemia on hematologic testing.