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

The levels, structure and composition of plasma lipoproteins were determined in 67 patients with homozygous beta-thalassaemia and compared to healthy or heterozygous members of the same families and to patients with either sickle cell or iron deficiency anaemia. Plasma total and LDL and HDL cholesterol levels were low in patients with homozygous beta-thalassaemia and with sickle cell anaemia. Plasma triglycerides did not differ between subjects. The low plasma and lipoprotein cholesterol was independent of age, transfusion requirements and splenectomy. Abnormal structure and composition of lipoproteins was found in homozygous beta-thalassaemia. The LDL was of higher density and was triglyceride-rich and cholesterol ester-poor. HDL separated to three populations. HDL2 was prominent (in spite of low plasma HDL cholesterol). HDL3 was of normal density and an intermediate HDL population, not found in normal subjects, was identified and designated HDL2-3. All three HDL populations were enriched with triglycerides and poor in cholesterol ester content. The modified LDL and HDL particles may then be possibly cleared rapidly from the plasma by activated monocytes and macrophages.
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PMID:Abnormal low and high density lipoproteins in homozygous beta-thalassaemia. 175 76

The two main causes of microcytic and hypochromic anaemia are iron deficiency and thalassaemia traits. Discriminant analysis based on a simple combination of classical red cell indices have been used to differentiate between iron deficiency anaemia and thalassaemia with varying degree of accuracy. Two new indices are now available from modern cell counters: red cell distribution width (RDW) and haemoglobin concentration distribution (HDW). Our discriminant analysis suggests that RBC, MCHC and RDW contribute significantly to the differentiation between iron deficiency anaemia and thalassaemia in both healthy donors and hospital-patient groups. In the discriminating process, previous workers have overlooked the heterogeneity of anaemia between anaemic groups as well as biological differences in MCV and MCH among the alpha and beta thalassaemia subjects. This study took into account of these biases and proved, for the first time, that differentiation between iron deficiency and thalassaemia by discriminant analysis was clinically reliable and not significantly biased by the severity of anaemia. The diagnostic accuracy of discriminant analysis was confirmed retrospectively by the reallocation algorithm using the jack-knife principle and prospectively by testing the discriminant functions on independent new samples. Selection of the red cell indices contributing to the discrimination of microcytic hypochromic anaemia was based on biological and statistical considerations. The clear separation of red cell index data of iron deficiency anaemia and thalassaemia traits was shown 3-dimensionally by surface plots.
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PMID:Discriminant analysis of iron deficiency anaemia and heterozygous thalassaemia traits: a 3-dimensional selection of red cell indices. 177 89

The perinatal outcome of 96 patients who had an antenatal haemoglobin value of less than 8.0 g/dl was compared with that of a similar number of controls who were matched for age and parity. Sixty-one patients (63%) had iron deficiency anaemia, 25 (26%) had alpha or beta thalassaemia minor, 7 (7.3%) had iron deficiency and thalassaemia trait, 2 had idiopathic pancytopenia and 1 had haemolytic anaemia due to systemic lupus erythematosus. Patients in the study group attended the antenatal booking clinic later, had less weight gain during pregnancy and their babies had lower birth-weights (2,984 g versus 3,177 g p less than 0.01) although there was no significant difference in the period of gestation at delivery. Six patients in the study group had placental abruption and another 2 patients had stillbirths but neither of these complications occurred in the control group. Although 37 patients (39%) in the study group received an antenatal blood transfusion, 53 (55%) of this group also had postnatal anaemia.
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PMID:A case controlled study of pregnancy complicated by severe maternal anaemia. 193 33

The authors studied the red cell distribution width (RDW) index (obtained by Coulter Counter S Plus IV) in normal subjects and in patients with beta-thalassaemia trait and iron deficiency anaemia. Statistics and reference limits for the above three conditions are given. In order to make a differential diagnosis between beta-thalassaemia trait and iron deficiency anaemia, linear discriminant analysis was carried out. Global correct classification was 91.5% on our first sample of patients and 88.8% on a subsequent validation sample of microcytic patients. The percent of correct beta-thalassaemia trait diagnoses was 94.4% and 86.7% for the first and validation samples respectively. For iron deficiency anaemia correct diagnoses of 86.2% and 90.9% were achieved.
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PMID:Relevance of red cell distribution width (RDW) in the differential diagnosis of microcytic anaemias. 193 25

Depending upon the transferrin saturation value, 214 serum samples were divided into three groups--iron overloaded (46 cases), iron deficient (61 cases) and normal (107 cases)--and tested with a micromethod based upon detection of unsaturated iron binding capacity. All the samples with iron overload could be distinguished from the other two groups, the results of the normal and iron deficient groups showing wide scatter and overlap. The high prevalence of iron deficiency anaemia and thalassaemia (iron overload) syndromes in India and other developing countries emphasises the need to differentiate these disorders at the earliest opportunity. The micromethod can be of immense help as it is a simple, rapid and inexpensive.
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PMID:Iron overload: detection using a micromethod for iron-binding capacity. 206 80

The haematological parameters of 97 cases of beta thalassaemia trait and 40 cases of delta beta thalassaemia trait have been compared. No differences in haemoglobin, haematocrit, MCV, MCH, ferritin, % saturation or free erythrocyte protoporphyrin have been found. The RDW, however, is significantly increased in delta beta thalassaemia trait, its mean value (+/- SD) being 20 (2.05), even higher than that found in iron deficiency anaemia. The discrimination function described by England and Fraser may be of help in distinguishing these entities.
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PMID:[Hematometric values in delta-beta thalassemia minor. Special importance of the erythrocyte distribution in comparison with beta thalassemia and iron deficiency]. 227 45

The mechanism(s) underlying the apparent resistance to malaria in certain inherited red cell disorders and iron deficiency anaemia remain poorly understood. The possibility that microcytic erythrocytes might inhibit parasite development, by physical restriction or reduced supply of nutrients, has been considered for many years, and never formally investigated. We sought to determine whether in vitro growth studies of P. falciparum could provide evidence to suggest that small red cell size contributes to malaria resistance in those red cell disorders in which microcytosis is a characteristic feature. Invasion and development of P. falciparum in iron deficient red cells (mean values for mean cell volume [MCV] 66 fl, mean cell haemoglobin [MCH] 19 pg) and in the red cells of two gene deletion forms of alpha-thalassaemia (mean MCV 71 fl, MCH 22 pg) were normal, assessed both morphologically, and by 3H-hypoxanthine incorporation. Although parasite appearances were normal in all cell types, morphological abnormalities were noted in iron deficient and thalassaemic cells parasitized by mature stages of P. falciparum, notably cellular ballooning and extreme hypochromia of the red cell cytoplasm. Using electron microscopy, the red cell cytoplasm in parasitized thalassaemic cells showed reduced electron density and abnormal reticulation. Normal invasion rates were observed following schizogony in microcytic cells of both types. Our findings indicate that whilst minor morphological abnormalities may be detected in parasitized iron deficiency and thalassaemic erythrocytes, development of P. falciparum in these conditions is not limited by small erythrocyte size.
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PMID:Unrestricted growth of Plasmodium falciparum in microcytic erythrocytes in iron deficiency and thalassaemia. 218 91

This paper describes the status of iron stores, the incidence and the hematological characteristics of iron deficiency anemia in children heterozygous for beta-thalassemia. In beta-thalassemia heterozygotes, iron stores were similar to the controls in infancy and tended to increase with age, reaching levels of moderate iron overload solely in adult males. Iron deficiency anemia occurred less frequently in children heterozygous for beta-thalassemia as compared to normal controls, while no difference between the two groups was observed in the incidence of iron deficiency. Ineffective erythropoiesis, typical of heterozygous beta-thalassemia, by causing an increase of iron absorption may limit the effect of iron shortage. At similar levels of iron depletion, however, children heterozygous for beta-thalassemia develop a more severe anemia as compared to non beta-thalassemic children. With the exception of two children, HbA2 levels in the presence of iron deficiency anemia remain in the range of heterozygous beta-thalassemia. In conclusion, our results indicate that children heterozygous for beta-thalassemia have normal iron stores but are relatively protected against the development of iron deficiency. When iron deficiency anemia develops, its clinical expression is usually more severe than in non beta-thalassemic children.
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PMID:Iron stores and iron deficiency anemia in children heterozygous for beta-thalassemia. 227 77

We analyzed the complete blood count (CBC) obtained from patients with various types of thalassemia in Thailand. As a control, samples from healthy donors and patients with iron deficiency anemia were also collected. Most cases of thalassemia could be differentiated from iron deficiency anemia by using a discrimination function equation. This screening method is applicable for mass-screening of patients with latent thalassemia in Thailand.
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PMID:Analysis of hematological data of thalassemia cases in Thailand. 261 54

A new expert system developed on a Macintosh personal computer using a commercially available artificial intelligence shell was compared with four different discriminant functions (DFs) for the differentiation of microcytic anemia into etiologic categories. Several databases were used with a different composition but all contained at least some samples from thalassemic individuals and from patients with iron deficiency anemia. The DFs analyzed were those proposed by England and Fraser, Green and colleagues, Mentzer, and by Shine and Lal. None of the databases performed satisfactorily when used singly, whereas very high false-positive rates were obtained by one of them. The diagnostic efficiency was somewhat improved by combining several DFs. An expert system using an artificial intelligence "shell" with an "interference engine" was developed using cluster analysis and a set of learning examples. The input necessary for the system to achieve a conclusion consists of MCV, RBC, and RDW as well as a statement as to whether the patient has anemia. Based upon the values of these parameters, the expert system will give an "advice" regarding the probabilities for thalassemia, iron deficiency, and/or other probabilities such as previous transfusions, anemia of chronic disease, laboratory error, etc. In a prospective trial, the system functioned with an accuracy of better than 85%.
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PMID:The use of inference strategies in the differential diagnosis of microcytic anemia. 262 97


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