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)

Fifty-three patients of thalassemia intermedia and 40 controls were studied for clinical evidence of thrombosis and laboratory evidence of hypercoagulable state. Thrombotic episodes were detected in 5 (9.4%) patients. Two of these 5 patients with thrombosis were splenectomized. Laboratory evaluation showed presence of thrombocytosis in 8 (15%), 5 of these were splenectomized. Platelet hyperaggregation was detected in 12 (22.2%) patients. Although rate of aggregation was slow in 7 (13.2%) patients, degree of aggregation was normal in these 7 patients and platelet hypoaggregation was not detected in any patient. Level of coagulation inhibitors protein C and protein S, and antithrombin III were decreased in 31 (58.4%) patients. There was no correlation between low level of protein C and protein S with hepatic dysfunction and iron overload. Antithrombin III level was decreased only in 8 (15%) patients. There was a statistically significant association between the lower level of this inhibitor and hepatic dysfunction. In conclusion, this study provides evidence for the existence of a chronic hypercoagulable state in patients with beta thalassemia intermedia, and suggests that expression of a procoagulant surface by thalassemia intermedia red blood cells may be the major underlying factor giving rise to platelet and coagulation inhibitor abnormalities in these patients. These alterations are not related to iron overload or hepatic dysfunction.
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PMID:Hypercoagulable state in five thalassemia intermedia patients. 1791 Nov 95

A high incidence of thromboembolic events have been reported among thalassemia intermedia patients especially in splenectomized patients. This study has been conducted to evaluate the coagulation state of patients referred to thalassemia research center at Booali Sina Teaching Hospital, Sari, Iran. This descriptive-cross sectional study was performed in 2007. Sixty thalassemia intermedia patients aged older than 10 years were enrolled. After recording demographic and therapeutic data of the participants, the plasma levels of Coagulant factors were measured. Obtained Data were analyzed using SPSS 13 software and t-test and chi-square tests. Of 60 studied patients, 62% were female. The mean age of the patients was 26.6 +/- 9.3 years. ASA tablet were used in 53.3%. None of the patients have experienced thromboembolic events. Decrease in protein C has been observed in 60%. Antithrombin III was decreased in 42% and protein S declined in 10% of the patients. Mutation in factor V leiden was seen in 3%. Splenectomy performed in 53.3% and there was no significant relation between low level of protein C and protein S, antithrombin III and presence of factor V Leiden with splenectomy (p > 0.05). Regarding to the high incidence of low levels of protein C, protein S and antithrombin III among thalassemia intermedia patients, there is an increased risk of thromboembolic events in the patients.
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PMID:Assessment of coagulation state and its related factors in thalassemia intermedia patients referred to thalassemia research center at Booali Sina Hospital Sari/IR Iran in 2007. 2097 99