Thalassemia major (homozygous beta‐thalassemia)

Logothetis, John; Constantoulakis, M.; Economidou, J.; Stefanis, Costas N.; Hakas, Pavlos; Augoustaki, Olga; Sofroniadou, K.; Loewenson, Ruth B.; Bilek, Mary K.

doi:10.1212/wnl.22.3.294

Cited by 95 publications

(72 citation statements)

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“…In 1972, Logothetis et al described a 'stroke syndrome' and neurological deficits compatible with transient ischemic attacks in about 20% of 138 cases of TM in Greece [43]. Similar evidence of transient ischemic attacks accompanied by a clinical picture of headache, seizures and hemiparesis was shown in 2.2% of patients with TM by Borgna Pignatti et al [37].…”

Section: Thrombosis In Thalassemiamentioning

confidence: 85%

Hypercoagulability in β-thalassemia: a status quo

Cappellini¹,

Poggiali

Taher

et al. 2012

Expert Review of Hematology

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Section: Thrombosis In Thalassemiamentioning

confidence: 85%

Hypercoagulability in β-thalassemia: a status quo

Cappellini¹,

Poggiali

Taher

et al. 2012

Expert Review of Hematology

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“…As early as 1972, Logothetis et al, 6 reviewing 138 cases of ␤-thalassemia major (␤-TM) in Greece, described a "stroke syndrome" in 2 patients and neurologic deficits compatible with transient ischemic attacks in about 20% of the cases. An Italian multicenter study of 735 patients with ␤-TM reported 16 individuals with cerebral thromboembolic events accompanied by a clinical picture of headache, seizures, and hemiparesis.…”

mentioning

confidence: 99%

The hypercoagulable state in thalassemia

Eldor

Rachmilewitz

2002

Blood

436

376

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Thalassemia is a congenital hemolytic disorder caused by a partial or complete deficiency of ␣-or ␤-globin chain synthesis. Homozygous carriers of ␤-globin gene defects suffer from severe anemia and other serious complications from early childhood. The disease is treated by chronic blood transfusion. However, this can cause severe iron overload resulting in progressive organ failure. Some forms of ␣ thalassemia are also associated with a similar clinical picture. Despite the difficulties associated with treatment, standards of care for thalassemic patients have improved in recent years, resulting in almost doubling of the average life expectancy. As a consequence, additional previously undescribed, complications are now being recognized. In particular, profound hemostatic changes have been observed in patients with ␤-thalassemia major (␤-TM) and ␤-thalassemia intermedia (␤-TI) and also in patients with ␣ thalassemia (hemoglobin H disease). The presence of a higher than normal incidence of thromboembolic events, mainly in ␤-TI, and the existence of prothrombotic hemostatic anomalies in the majority of the patients, even from a very young age, have led to the recognition of the existence of a chronic hypercoagulable state in thalassemic patients. Despite the appearance of numerous publications on the frequent occurrence of thromboembolic complications in thalassemia, this complication has not been emphasized or comprehensively reviewed. This review summarizes the current literature and discusses possible mechanisms of the lifelong hypercoagulable state that exists in thalassemia. (Blood. 2002;99:36-43)

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“…Another patient presented with headache, visual blurring, behavioral changes, and right hemiparesis shortly after a blood transfusion, and the clinical picture was that of the posttransfusion syndrome. ever, recurrent transient cerebral symptoms such as dizziness, visual blurring, and fainting were reported in 20% of the 138 patients in the series of Logothetis et al, 2 and these symptoms were presumed to be ischemic in nature since they improved following transfusion.…”

Section: Casementioning

confidence: 98%