MR imaging of the brain: findings in asymptomatic patients with thalassemia intermedia and sickle cell-thalassemia disease.

Manfrè, L.; Giarratano, E; Maggio, Aurelio; Banco, A; Vaccaro, Gina M.; Lagalla, Roberto

doi:10.2214/ajr.173.6.10584785

Cited by 73 publications

(96 citation statements)

References 21 publications

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“…20,21 These thrombi could contribute to the pulmonary hypertension, low lung capacity, hypoxemia, and diffusion defects associated with right heart failure (cor pulmonale) [22][23][24][25][26][27] and to the high frequency of ischemic brain lesions associated with asymptomatic brain damage as detected by MRI. 14 Several etiologic factors may play a role in the pathogenesis of the hypercoagulable state in thalassemia. The specific changes in the lipid membrane composition of the abnormal RBCs and the hemosiderosis may contribute to the activation of the coagulation process and the activation of other blood cells, including the platelets, monocytes, and granulocytes, alone or together, and may induce activation of the vascular endothelium, which further contributes to the thrombotic process.…”

Section: Discussionmentioning

confidence: 99%

“…[10][11][12][13] Asymptomatic brain damage has also been reported; results from magnetic resonance imaging (MRI) on 41 patients with ␤-thalassemia intermedia (␤-TI) revealed asymptomatic brain damage including ischemic lesions as a frequent occurrence affecting 37% of patients. 14 Damage was inversely correlated with hemoglobin levels in patients with ␤-TI and increased with age.…”

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confidence: 93%

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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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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 93%

The hypercoagulable state in thalassemia

Eldor

Rachmilewitz

2002

Blood

436

376

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“…15 SCI is most common in patients with hemoglobin SS but may also be identified in individuals with heterozygous genotypes including sickle ␤-thalassemia and hemoglobin SC (HbSC) disease. Approximately 3% to 38% of patients with sickle ␤-thalassemia, 17,18,20,21 and 5% to 31% of those with HbSC disease, 15,22 will have SCI. However, limited sample size allows no significant inferences about the rate of progression or potential benefit of therapy.…”

Section: Clinical Significance Of Scismentioning

confidence: 99%

Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia

DeBaun

Armstrong

McKinstry

et al. 2012

Blood

281

292

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Silent cerebral infarct (SCI) is the most common form of neurologic disease in children with sickle cell anemia (SCA). SCI is defined as abnormal magnetic resonance imaging (MRI) of the brain in the setting of a normal neurologic examination without a history or physical findings associated with an overt stroke. SCI occurs in 27% of this population before their sixth, and 37% by their 14th birthdays. In adults with SCA, the clinical history of SCI is poorly defined, although recent evidence suggests that they too may have ongoing risk of progressive injury. Risk factors for SCI include male sex, lower baseline hemoglobin concentration, higher baseline systolic blood pressure, and previous seizures. Specific morbidity associated with SCI includes a decrement in general intellectual abilities, poor academic achievement, progression to overt stroke, and progressive SCI. In addition, children with previous stroke continue to have both overt strokes and new SCI despite receiving regular blood transfusion therapy for secondary stroke prevention. Studies that only include overt stroke as a measure of CNS injury significantly underestimate the total cerebral injury burden in this population. In this review, we describe the epidemiology, natural history, morbidity, medical management, and potential therapeutic options for SCI in SCA. IntroductionOne of the most devastating medical complications of sickle cell anemia (SCA) is cerebral injury, which limits the full potential of the developing child and adult. The most common neurologic injury in SCA is silent cerebral infarct (SCI). Although there are few studies, SCI occurs in approximately one-quarter of children with SCA before their sixth birthday 1 and approximately one-third before their 14th birthday. 2 The first evidence that SCI had clinical significance was published in a cross-sectional computed tomography (CT) scan study of 25 patients. Five had cerebral infarcts but only 4 had a focal neurologic deficit (overt stroke). 3 The investigators referred to the fifth patient as having a covert stroke, 3 a term also used by others. 4 The term SCI or silent stroke has gained widespread use to describe this condition because the Cooperative Study of Sickle Cell Disease (CSSCD) used standard clinical magnetic resonance imaging (MRI) to define the radiologic parameters. 5 Subsequently, only a few studies of SCI have been completed. Until recently, the clinical importance of identifying SCI did not seem to justify the expense of MRI requiring sedation or anesthesia in preschool children, particularly in the absence of an effective intervention. Despite being the most common type of cerebral injury among children and adults with SCA, relatively little is known about the cause and optimal therapy of SCI. We will review the current literature on definition, epidemiology, natural history, clinical significance, medical management, and potential therapeutic options for the treatment of SCI in SCA. Definition of SCI in children with SCAThe largest longitudinal cohort study to a...

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“…Although overt stroke occurs more frequently in TM than in TI (28 vs 9%, respectively) due to a higher prevalence of conventional stroke-related risk factors (diabetes mellitus, heart failure and arrythmias) [39,44,45], a high prevalence of silent strokes in TI patients has been documented [46]. The earliest study was conducted in 1999 and showed a 37.5% rate of ischemic lesions on brain MRI in 16 patients with TI who were neurologically intact and had no conventional stroke-related risk factors [47]. More recently, a cross-sectional brain MRI study was conducted in Lebanon on 30 splenectomized adults with TI, who were selected from a larger cohort of patients based on absence of neurological or gross cognitive signs or symptoms and any stroke-related risk factors.…”

Section: Thrombosis In Thalassemiamentioning

confidence: 99%