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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a retrospective study the diagnostic value of erythrocyte zinc protoporphyrin (ZPP) measurement as a means of distinguishing iron deficiency anemia from thalassemia syndromes in patients with microcytosis was explored. ZPP values were increased in all patients with iron deficiency and in part of the patients with thalassemia. The combined measurement of erythrocyte mean corpuscular volume (MCV) and ZPP resulted in a correct classification of patients with iron deficiency and with thalassemia in more than 95%. The predictive value of this method is better than the results obtained by using formulae derived from red cell indices. In population screening programs for thalassemia syndromes, in which MCV determination is used as the initial test, the ZPP test is recommended as a second test, in order to discriminate between patients with microcytosis due to iron deficiency and patients with microcytosis due to thalassemia syndromes.
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PMID:Combined use of erythrocyte zinc protoporphyrin and mean corpuscular volume in differentiation of thalassemia from iron deficiency anemia. 957 78

Of the uncommon anemias, "common" types include the anemia of renal disease, thalassemia, myelodysplastic syndrome and the anemia of chronic disease. These conditions may be suggested by the clinical presentation, laboratory test values and peripheral blood smear, or by failure of the anemia to respond to iron supplements or nutrient replacement. The principal cause of the anemia of renal disease is a decreased production of red blood cells related to a relative deficiency of erythropoietin. When treatment is required, erythropoietin is administered, often with iron supplementation. In the anemia of chronic disease, impaired iron transport decreases red blood cell production. Treatment is predominantly directed at the underlying condition. Since iron stores are usually normal, iron administration is not beneficial. Thalassemia minor results from a congenital abnormality of hemoglobin synthesis. The disorder may masquerade as mild iron deficiency anemia, but iron therapy and transfusions are often not indicated. In the myelodysplastic syndrome, blood cell components fail to mature, and the condition may progress to acute nonlymphocytic leukemia. The rate of progression depends on the subtype of myelodysplasia, but the leukemia is usually resistant to therapy.
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PMID:'Common' uncommon anemias. 1006 9

The effect of iron deficiency anaemia (IDA) on CD71 expression by peripheral blood lymphocytes was studied in 43 children with iron deficiency anaemia. 18 healthy age-matched children were selected as the control group. 11 children with beta-thalassaemia trait were also studied. Lymphocytes bearing CD71 were enumerated by flow cytometric analysis of peripheral blood. At diagnosis, CD71+ peripheral lymphocytes (mean+/-SE) was 5.90+/-0-76% in patients with IDA and 12.60+/-0.98% in healthy controls (P=0.000). In beta-thalassaemia trait patients the peripheral blood CD71+ lymphocytes were 7.80+/-1.20%. In IDA patients there was a statistically significant correlation between the levels of CD71+ peripheral lymphocytes and haemoglobin value (P = 0.000). In 19 patients studied at days 0 and 30 of oral iron therapy, the number of peripheral blood CD71+ lymphocytes was shown to be increased from 5.90+/-0.76% to 12.11+/-1.21%. In severe IDA presence of a limited number of CD71+ peripheral blood lymphocytes indicated that severe IDA should be borne in mind when considering conditions responsible for the suppression of lymphocyte proliferation.
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PMID:Transferrin receptor on peripheral blood lymphocytes in iron deficiency anaemia. 1008 85

Iron deficiency modulates the synthesis of HbA2, resulting in reduced HbA2 levels in patients with iron deficiency anaemia. The diagnosis heterozygous beta-thalassaemia is based on a raised HbA2 level. Patients with beta-thalassaemia and concomitant iron deficiency can show normal HbA2 levels. It is of clinical importance to know the quantitative effect of iron-deficient erythropoiesis on the levels of HbA2 in order to be able to determine which iron-deficient patients with normal HbA2 levels have to be retested after iron therapy in thalassaemia screening programmes. In this study, HbA2 levels in 150 patients with iron-deficiency anaemia and 71 healthy controls have been measured. A linear correlation is found in the patient group between HbA2 and Hb, HbA2 and MCV, and HbA2 and erythrocyte zinc protoporphyrin (ZPP). In future studies, the correlation between HbA2 and erythrocyte parameters in patients with heterozygous beta-thalassaemia and concomitant iron deficiency has to be examined. We recommend that ZPP be measured in these studies too, as ZPP levels may be a better indicator of concomitant iron deficiency than Hb or MCV in thalassaemic patients.
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PMID:Influence of iron deficiency anaemia on haemoglobin A2 levels: possible consequences for beta-thalassaemia screening. 1020 99

The most widely used discriminant functions and red cell indices for differential diagnosis of thalassemia traits from iron deficiency anemia were evaluated for their abilities to identify HbE-containing blood samples. The functions were as follows: F1 = 0.01 x MCH x (MCV)2; F2 = RDW x MCH x (MCV)2/Hb x 100; F3 = MCV/RBC; and F4 = MCH/RBC. Other red cell parameters including RDW, hemoglobin content, mean cell volume, mean cell hemoglobin as well as red cell counts, were also evaluated to distinguish HbE from the normal population. Hemoglobin electrophoresis was used as a confirmatory test. The results showed that F1, F2 and F3 as well as other red cell parameters of HbE-containing samples were different from those of HbA2A-containing red cells although there was no statistical significance. However, F4 and MCHC showed no difference between the two groups. It can be concluded from the present study that identification of hemoglobin E especially the heterozygous form by using parameters from an electronic cell counter is not easy. Discriminant functions and red cell indicies might be used as an initial diagnosis. But confirmation is needed in all cases. Applying the MCV of 80 fl will miss 5 per cent of hemoglobin E carrier but will not miss the homozygous form.
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PMID:Correlation between some discrimination functions and hemoglobin E. 1080 80

A retrospective study of 126 patients with extreme thrombocytosis (defined as a platelet count > or = 1,000 x 10(9)/L) was performed during a five-year period (June 1994-June 1999). The aim of this study was to determine the etiology and to evaluate the clinical consequences of extreme thrombocytosis. Seventy patients (55.5%) had reactive thrombocytosis (RT) with an age range of 43 +/- 2.2 years, 56 (44.5%) had chronic myeloproliferative disorders (MPD) with an age range of 53 +/- 2.4 years. Underlying causes of RT were malignancy (25/70 or 35.7%), infection (16/70 or 22.9%), postsplenectomized beta-thalassemia/Hb E (11/70 or 15.7%), inflammation (12/70 or 17.1%), iron deficiency anemia (6/70 or 8.6%). Duration post splenectomy in our beta-thalassemia/Hb E patients ranged from 4 months to 21 years, with a median of 10 years. Subtypes of our MPD cases were chronic myeloid leukemia (30/56 or 53.6%), essential thrombocytosis (18/56 or 32.1%), polycythemia vera (4/56 or 7.1%), agnogenic myeloid metaplasia (3/56 or 5.4%) and unclassified MPD (1/56 or 1.8%). Bleeding and thrombotic tendency were respectively noted in 7 (12.5%) and 2 (3.6%) of MPD patients. Two patients of the MPD group (3.6%) experienced both bleeding and thrombotic episodes. One patient (1.4%) of the RT group developed vasculitis-associated thrombosis. However, none of the patients in the RT group had bleeding complications. Extreme thrombocytosis was not a rare condition in a university hospital population, and bleeding and/or thrombotic complication was more common in the MPD group.
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PMID:Etiology and incidence of thrombotic and hemorrhagic disorders in Thai patients with extreme thrombocytosis. 1086 14

In Thailand, there are around 2,000 clinical laboratories in private and government hospitals, By the end of year 2004, all of these laboratories are required to use the same or comparable standard nationwide. Many laboratories are in the process of starting ISO/IEC Guide 25 for the fulfillment of laboratory accreditation. To run the standard system of hematology laboratories in Thailand, we have considered three main aspects: standard in process, method selection and academic interpretation. Because of the wide spectrum of blood diseases in Thailand: thalassemia, iron deficiency anemia and G6PD deficiency hemolytic disease, the analysis and interpretation of laboratory results using different technology are of great importance. National plan has thus set up in two direction, one for standard process and another for academic approach.
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PMID:Plan and process for hematology laboratory standard in Thailand. 1092 79

The frequency and etiology of anemia were investigated in 2,913 children between six and 16 years of age in Sanliurfa, in the southeast region of Turkey. Anemia was found in 142 (7.8%) children in the 6-11 years of age group, and in 16 (1.5%) in the 12-16 years of age group; in total, in 158 (5.4%) children. Causes were iron deficiency in 93 (58.9%) children, beta-thalassemia heterozygosity in 10 (6.3%) children, chronic disease that causes anemia of inflammation in 30 (19.0%) children, and intestinal parasitic infections in 17 (10.8%) children. In eight (5.1%) children, the cause of anemia could not be determined. The study's results showed that iron deficiency anemia and chronic and parasitic disease are important problems in schoolchildren of Sanliurfa, while beta-thalassemia and hemoglobinopathies have less importance.
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PMID:The frequency and etiology of anemia among children 6-16 years of age in the southeast region of Turkey. 1093 71

Autoxidation of globin chains and iron overload are the suggested mechanisms for the increased oxidative stress in beta-thalassemia. The aim of this study was to evaluate the extend of lipid peroxidation and antioxidant status of patients with beta-thalassemia and iron deficiency anemia (IDA) and compare the results with healthy subjects. Oxidant and antioxidant status of the children with beta-thalassemia major (n = 22) and iron deficiency anemia (n = 19) were studied. Healthy controls (n = 14) were age and sex matched. Fresh anticoagulated venous blood samples were obtained from all children. Conjugated diene (CD) and thiobarbituric acid-reactive (TBARS) substances were analyzed to indicate the oxidative parameters, whereas the erythrocyte superoxide dismutase (SOD) and glutathione peroxidase (GPx) were measured to show the antioxidant status of the children. Plasma TBARS and CD concentrations were elevated in beta-thalassemia compared to IDA. When compared to the controls, elevation in TBARS was significant. In the iron-deficiency group both TBARS and CD levels were decreased compared to the controls. SOD and GPx activities were increased in the beta-thalassemia group. SOD in beta-thalassemia was higher than both IDA and the controls and GPx activity was higher than the IDA group. In vivo lipid peroxidation was increased in children with beta-thalassemia major. This leads to a compensatory increase in antioxidant enzymes, whereas IDA does not lead to lipid peroxidation with a normal antioxidant enzyme activity.
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PMID:Lipid peroxidation and antioxidant status in beta-thalassemia. 1112 1

Earlier studies show that in iron deficiency with anaemia and in latent iron deficiency neurotransmitters are altered. The changes induced in the fetal brain are irreversible on rehabilitation. The important alterations in glutamate metabolism in latent iron deficiency stimulated studies on gamma aminobutyric acid and glutaminate receptors. It was observed that binding of 3H-muscimol at pH 7.5 and 1 mg protein/assay increased significantly in synaptic vesicular membranes and under similar conditions 3H-glutamate binding showed reduction. Thus iron deficiency played a role in both excitatory and inhibitory neurotransmitter receptors. To elucidate the role of body iron status on the brain, anaemic children with thalassemia and iron deficiency were subjected to 'magnetic resonance spectroscopy' of globus pallidus, caudate and dentate nuclei and there was no change in iron content. The concentrations of creatinine and aspartate increased, with lowering of choline content. The findings were similar in thalassemia as well as iron deficiency anaemia, suggesting that in anaemia changes operate through reduced oxygen availability.
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PMID:Iron and the brain: neurotransmitter receptors and magnetic resonance spectroscopy. 1150 3


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