Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The in vitro immune functions of peripheral blood lymphocytes have been studied in 12 children with beta-thalassaemia major and hypersplenism. The study was performed prior to splenectomy and on the 2nd, 6th, 15th and 30th day after splenectomy. It was found that before splenectomy, patients had low numbers of blood leucocytes, normal rosette and T3 lymphocyte counts, low T4 and normal T8 lymphocyte counts with a T4/T8 ratio below 2, impaired T lymphocyte mitogenic responses induced by PHA, increased numbers of polyvalent and monovalent B lymphocytes and normal immunoglobulin levels of IgG and IgA. After splenectomy, especially on the 2nd day, leucocytosis, a significant decrease of T cells and their subsets and a reduction of the IgM levels were found. These parameters, except the IgM levels, increased until the 30th day after splenectomy and reached presplenectomy values. On the 2nd day after splenectomy, large mononuclear cells behaving like immunocytes appeared in the peripheral blood. They had the phenotype of T3, T4, T8, B lymphocytes and OKM1 monocytes. All the large mononuclear cells increased significantly on the 6th day after splenectomy and remained elevated during the whole study (30 days). The T4/T8 ratio was increased, but no increase was found in the functional responses of T lymphocytes.
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PMID:Immunological profile after splenectomy in children with beta-thalassaemia major. 312 90

Iron toxicity in beta-thalassemia major is the main cause of oxidative stress and cell mediated immune deficiencies. Despite indicative signs of severe oxidative deficiencies associated with beta-thalassemia major, such as decreased level of plasma antioxidants and depletion of erythrocyte glutathione, little is known about intracellular redox status of immune cells. Since glutathione is a primary intracellular antioxidant and plays an essential role in several functions in T cells, in this study intracellular glutathione (GSH) levels as well as proliferation of PHA-activated peripheral blood mononuclear cells (PBMC) were investigated in 28 beta-thalassemia major patients and 28 healthy age-matched individuals. Considering the potential benefits of flavonoids in the therapy of oxidative stress, the effects of silymarin on the GSH levels and proliferation of PBMC from normal and thalassemia individuals were further examined. Quantitative determination of intracellular GSH and proliferative response of PBMC to PHA were performed before and after 72 h incubation of PBMC with various concentrations of silymarin (0, 5, 10, or 20 mug/ml). Results demonstrated a significant reduction of GSH and proliferation in beta-thalassemia major cells; however treatment with silymarin led to restoration of both GSH levels and PBMC proliferation in thalassemia patients. Considerably low levels of GSH and depressed proliferative response of PBMC in beta-thalassemia major may be responsible for the cell mediated immune abnormalities in iron overload conditions. Moreover, the GSH restoration and improvement of PBMC growth by silymarin is a possible explanation for its recently reported antioxidant and immunostimulatory activities. These data suggest the benefit of using flavonoids to normalize immune dysfunction in beta-thalassemia major. The immunomodulatory effects of silymarin in beta-thalassemia major are currently under further investigation in a double blind clinical trial.
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PMID:Effects of silymarin on the proliferation and glutathione levels of peripheral blood mononuclear cells from beta-thalassemia major patients. 1678 43