High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease

Wang, Winfred C.; Kovnar, Edward H.; Tonkin, Ina L.D.; Mulhern, Raymond K.; Langston, J. William; Day, Sara W.; Schell, Michael J.; Wilimas, Judith A.

doi:10.1016/s0022-3476(05)82150-x

Cited by 168 publications

(80 citation statements)

References 12 publications

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“…1 Chronic transfusions are effective in preventing the short-term recurrence of infarctive stroke 2 and the occurrence of a first cerebral infarction in HbSS children who have abnormally high velocities on transcranial Doppler ultrasonography (TCD). 3 After acute stroke in sickle cell anemia patients, chronic transfusion therapy has been shown to nearly stop progression of stenosis in most children, 4,5 but progressive large vessel disease has been evidenced in HbSS children presenting with moyamoya syndrome.…”

Section: Introductionmentioning

confidence: 99%

Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia

Bader-Meunier¹,

Verlhac²,

Elmaleh‐Bergès³

et al. 2009

Haematologica

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This retrospective study assessed the long-term effect of transfusional exchange therapy on MRA/MRI abnormalities in 24 homozygous sickle-cell anemia (HbSS) children presenting with abnormal brain MRA. The median time elapsed from baseline to last available MRA was 29 months. Follow-up MRAs showed improvement, stabilization or worsening of cerebrovascular lesions in 11, 6 and 7 patients respectively. Complete normalization of MRA was observed in 6 patients within a mean time of 1.4 years, but stenosis recurred at the same location in the 4 patients in whom transfusion therapy was discontinued. Baseline severe stenosis/occlusion of large cerebral arteries and occurrence of moyamoya syndrome were significantly associated with an absence of improvement of the cerebral vasculopathy. These data emphasize the heterogeneity of the course of cerebrovasculopathy in SS children receiving chronic transfusion. Further studies are needed to determine whether different therapeutic approaches have to be considered according to these different evolutive patterns in SS children.Key words: sickle-cell anemia, children, cerebral vasculopathy, stroke.

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Section: Introductionmentioning

confidence: 99%

Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia

Bader-Meunier¹,

Verlhac²,

Elmaleh‐Bergès³

et al. 2009

Haematologica

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“…- 4 Possible iatrogenic complications include blood-borne transmitted disease, alloimmunization, and iron overload. A less aggressive transfusion regimen, allowing HbS to rise to 50% between transfusions, has been proposed as equally efficacious in preventing stroke recurrence.…”

mentioning

confidence: 99%

Effects of total hemoglobin and hemoglobin S concentration on cerebral blood flow during transfusion therapy to prevent stroke in sickle cell disease.

Hurlet‐Jensen

Prohovnik

Pavlakis

et al. 1994

Stroke

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The standard treatment of stroke in sickle cell disease is chronic transfusion to maintain the fraction of abnormal hemoglobin (hemoglobin S [HbS]) below 20%. Risks associated with such transfusion can be reduced by allowing higher HbS levels, but the physiological consequences of this modification are unknown. Cerebral blood flow is elevated in sickle cell disease proportionate to the degree of anemia and is reduced by transfusion. We tested the effects of various HbS levels on cerebral blood flow during the course of transfusion therapy. We monitored cerebral blood flow (by the 133Xe inhalation method) in three patients whose chronic transfusion program was changed from a traditional regimen (HbS < 20%) to a moderate one, allowing HbS to rise to 45% to 50% between treatments. As expected, cerebral blood flow was higher with lower hemoglobin and higher HbS concentration. However, the HbS fraction appeared to exert a separate influence on the hyperemia, independent of total hemoglobin concentration. Furthermore, cerebral blood flow was higher during the modified regimen, despite equivalent anemia. These results suggest caution in adapting the modified transfusion regimen. Although HbS concentrations of 50% did not cause any frank neurological sequelae, the possible consequences of the associated hyperemia over time are unknown. We conclude that larger clinical and physiological studies of moderate transfusion regimens (allowing higher concentration of HbS) are necessary before it can become standard therapy.

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“…The US National Heart, Lung, and Blood Institute and UK National Health Service recommend all children should have TCD screening and be transfused if their velocities are greater than 200cm/s [46]. Current guidelines state that those children should be transfused indefinitely [47], but the TCD with transfusions changing to hydroxyurea (TWiTCH) trial suggests that, for those with no MRA abnormality may be able to switch to hydroxyurea prophylaxis after a year of transfusion [48]. Hydroxyurea does appear to reduce TCD velocities even without prior blood transfusion [1]; so, in settings where TCD is available but blood transfusion is not possible or is considered hazardous, it is probably reasonable to start hydroxyurea while the results of controlled trials are awaited [49].…”

Section: Primary and Secondary Stroke Preventionmentioning

confidence: 99%

Neurological Complications and MRI

Kawadler¹,

Kirkham²

2016

Sickle Cell Disease - Pain and Common Chronic Complications

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Cerebrovascular diseases (cerebral infarction, intracranial haemorrhage and vasculopathy) are common manifestations of sickle cell disease (SCD) associated with significant morbidity and mortality. These neurological complications and potential corresponding neuropsychological compromise may have devastating consequences for a child with SCD. This chapter aims to review the neurological complications in SCD using magnetic resonance imaging (MRI) as both a qualitative and a quantitative tool for detecting abnormality. Advanced MRI pulse sequences, such as high-resolution 3D T1-weighted imaging for brain volumetrics, diffusion tensor imaging for white matter integrity and non-invasive perfusion MRI for cerebral blood flow (CBF) measurement, can provide additional information about the structure and function of brain tissue beyond the scope of conventional clinical imaging. These studies have set to establish quantitative biomarkers that relate to disease severity and neuropsychological sequelae.

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High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease

Cited by 168 publications

References 12 publications

Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia

Effect of transfusion therapy on cerebral vasculopathy in children with sickle-cell anemia

Effects of total hemoglobin and hemoglobin S concentration on cerebral blood flow during transfusion therapy to prevent stroke in sickle cell disease.

Neurological Complications and MRI

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