Neurological complications of beta-thalassaemia major.

Sinniah, D.; Vignaendra, Velupillay; Ahmad, Kaisar

doi:10.1136/adc.52.12.977

Cited by 40 publications

(28 citation statements)

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“…Clinical studies and autopsy findings indicate that bthalassaemia major (TM) patients suffer from thromboembolic events, including cerebrovascular accidents (Logothetis et al, 1972), transient ischaemic attacks (Sinniah et al, 1972;Paolini et al, 1983;Wong et al, 1990), pulmonary embolism (Sonakul et al, 1990;Sonakul & Fucharoen, 1992;Sumiyoshi et al, 1992), renal vein thrombosis and deep vein thrombosis (Michaeli et al, 1992). Several platelet and coagulation anomalies, frequently observed in TM or thalassaemia intermedia (TI) patients, suggested the existence of a hypercoagulable state.…”

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confidence: 99%

In‐vivo platelet activation correlates with red cell anionic phospholipid exposure in patients with β‐thalassaemia major

et al. 1997

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Summary. Several clinical and laboratory findings suggest the presence of a chronic hypercoagulable state in patients with b-thalassaemia major (TM). We have previously shown that isolated TM red blood cells (RBC) strongly enhance prothrombin activation, suggesting an increased membrane exposure of procoagulant phospholipids (i.e. phosphatidylserine). In this study we quantitated the procoagulant activity of RBC in TM and thalassaemia intermedia (TI) patients. We also determined the fraction of activated platelets expressing p-selectin (CD62p) or CD63 in these subjects. Both assays were performed by dual-colour flow cytometry. A significantly (P < 0 . 01) higher fraction of FITCannexin V-labelled RBC was found in TM and TI patients, compared to the controls. A highly significant correlation (P < 0 . 001) was found in TM patients between the number of RBC-bound annexin V molecules and the fraction of CD62p (p-selectin) or CD63-positive platelets. This association between annexin V binding to TM RBC and the expression of platelet activation markers was also found in individual TM patients over time. Thus, the procoagulant surface of TM RBC may accelarate thrombin generation in vivo which, in turn, triggers platelet activation.

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In‐vivo platelet activation correlates with red cell anionic phospholipid exposure in patients with β‐thalassaemia major

et al. 1997

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“…11 Pengetahuan mengenai hubungan fungsi kognitif pada penyandang thalassemia dengan jenis kelamin, keadaan hipoksia kronik, onset transfusi, dan keadaan hemosiderosis sampai saat ini masih meragukan. 12,13 Pemeriksaan fungsi kognitif pada penyandang thalassemia seharusnya dilakukan secara rutin sehingga apabila dicurigai atau didapatkan adanya gangguan kognitif, dapat segera diberikan intervensi. 7 Penapisan fungsi kognitif dengan pemeriksaan status mini mental (MMSE) dapat digunakan klinik.…”

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