Stroke in patients with sickle cell disease

Webb, Jennifer; Kwiatkowski, Janet L.

doi:10.1586/ehm.13.25

Cited by 50 publications

(66 citation statements)

References 166 publications

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“…Clinical ischemic strokes in patients with SCD are associated with prior transient ischemic attack, increased systolic blood pressure, acute coronary syndrome, prior silent infarcts, and nocturnal hypoxemia. 4 However, hemorrhagic strokes are associated with older age, low steady state hemoglobin, low steady state leukocytes count, 3 transfusion within 2 weeks, and treatment with corticosteroids or nonsteroidal anti-inflammatory drugs within 2 weeks. 4 Silent infarcts are associated with leukocytosis, seizures, lower baseline hemoglobin, male sex, and hypertension.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…4 However, hemorrhagic strokes are associated with older age, low steady state hemoglobin, low steady state leukocytes count, 3 transfusion within 2 weeks, and treatment with corticosteroids or nonsteroidal anti-inflammatory drugs within 2 weeks. 4 Silent infarcts are associated with leukocytosis, seizures, lower baseline hemoglobin, male sex, and hypertension. 4 Clinically apparent ischemic stroke, hemorrhagic stroke, and clinically silent infarcts are all increased in SCD.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…4 Silent infarcts are associated with leukocytosis, seizures, lower baseline hemoglobin, male sex, and hypertension. 4 Clinically apparent ischemic stroke, hemorrhagic stroke, and clinically silent infarcts are all increased in SCD. Ischemic strokes are secondary to vasculopathy and intracranial large artery stenosis.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Diminished reserve and impaired cerebral perfusion pressure likely contribute to infarction. 4 Moyamoya syndrome is present in 20% to 30% of patients with SCD undergoing cerebral angiogram 5 and predisposes to both ischemic stroke and intracranial hemorrhage. Moyamoya disease is idiopathic, progressive stenosis of the terminal internal carotid arteries and their main branches.…”

Section: Talahma Et Al Sickle Cell Disease and Stroke E99mentioning

confidence: 99%

“…4 Clinically silent infarction is defined as increased T2 signal abnormalities on MRI without corresponding deficits. Twenty-two percentage of children with SCD between 6 and 19 years of age have silent infarcts, usually involving small vessels in watershed distributions.…”

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Sickle Cell Disease and Stroke

Talahma

Strbian

Sundararajan

2014

Stroke

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Downloaded from Talahma et al Sickle Cell Disease and Stroke e99hemorrhages in patients with SCD are associated with aneurysms, which tend to be multiple, and rupture despite smaller size. 4 Clinically silent infarction is defined as increased T2 signal abnormalities on MRI without corresponding deficits. Twenty-two percentage of children with SCD between 6 and 19 years of age have silent infarcts, usually involving small vessels in watershed distributions. Diminished reserve and impaired cerebral perfusion pressure likely contribute to infarction. 4 Moyamoya syndrome is present in 20% to 30% of patients with SCD undergoing cerebral angiogram 5 and predisposes to both ischemic stroke and intracranial hemorrhage. Moyamoya disease is idiopathic, progressive stenosis of the terminal internal carotid arteries and their main branches. A compensatory collateral network develops at the base of the brain.5 On catheter angiography, this network appears as a puff of smoke or moyamoya in Japanese. Moyamoya syndrome describes radiographic features similar to idiopathic moyamoya disease that may coexist with other conditions such as SCD. ManagementStroke symptoms in SCD are similar to those in other patients. Patients should be evaluated for traditional risk factors that may coexist with SCD. Vascular imaging should be obtained to evaluate for aneurysms or moyamoya. In the case of hemorrhage, if computed tomographic angiography/magnetic resonance angiography is unrevealing, conventional angiography should be performed. Importantly, hyperosmolar intravenous contrast may increase the risk of ischemic stroke. Patients should be pretreated with intravenous hydration and transfusion to maintain the HbS% <20 to 50 before iodinated contrast. 6In addition to standard stroke care, patients with SCD and acute stroke should receive transfusion with a goal of decreasing the HbS% to <30.2 With simple transfusion, patients do not have their own blood removed but receive transfused blood. In exchange transfusion, the patient's blood is removed and replaced with an equal volume of packed RBCs. Exchange transfusion can be either manual, in which whole blood is replaced with donor blood, or automated. In erythrocytapheresis, sickled RBCs are identified and removed by automated centrifugation and replaced with donor packed RBCs. A retrospective study suggests that exchange transfusion at the time of presentation is associated with lower stroke recurrence compared with simple transfusion.7 Blood products should be thoroughly matched for minor antigens, and leukocyte-filtered blood should be used. Importantly, packed RBCs should be screened for sickle hemoglobin. Unfortunately, recurrent transfusions increase the risk of iron overload, allergic reactions, infections, alloimmunization, and transfusion reactions.The role of thrombolysis in patients with SCD is unclear. To our knowledge, there are no published data on thrombolytic therapy in patients with SCD. Most experts recommend that thrombolysis be considered in patients meeting criteria. The ...

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Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Talahma Et Al Sickle Cell Disease and Stroke E99mentioning

confidence: 99%

mentioning

confidence: 99%

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Sickle Cell Disease and Stroke

Talahma

Strbian

Sundararajan

2014

Stroke

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Stroke in sickle cell patients, epidemiology, pathophysiology, systemic and surgical treatment options and prevention strategies

Shah,

Alberts,

Ngo

et al. 2024

Clinical and Translational Dis

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BackgroundA hereditary haemoglobinopathy known as sickle cell disease (SCD) affects over 100 000 people in the United States severely. Cerebrovascular disease is a prominent consequence of SCD. By the age of 30, 53% of patients have silent cerebral infarcts (SCIs) (a stroke that occurs without any obvious symptoms because it damages a small part of the brain that isn't responsible for any essential functions), and by the age of 40, 3.8% have overt strokes.Main bodyThe multidimensional burden of cerebrovascular illness in SCD is reviewed in detail in this article, which includes both clinical strokes and the frequently asymptomatic SCIs. The intricate pathophysiology of SCD and stroke is explored. With SCD, there are currently very few methods for preventing primary and secondary stroke; the most common ones are hydroxyurea and blood transfusion. Nevertheless, not enough research has been done on the possible contributions of anticoagulation and aspirin to strokes linked to SCD. Promising evidence is also highlighted in the study, suggesting that new drugs intended to treat SCD may be able to alleviate leg ulcers and renal impairment in addition to reducing unusually high transcranial Doppler flow velocity – a crucial component of cerebrovascular events. Given that these novel medications specifically target haemolysis and vaso‐occlusion, the two main causes of strokes in this population, more research is desperately needed to determine whether they are effective in avoiding strokes in people with SCD. The literature review also emphasizes how common healthcare inequities are and how they hinder advancements in SCD research and management in the United States.ConclusionTo successfully address these inequities, the evaluation recommends more funding for both SCD management and research, as well as for patient and clinician education. This multimodal viewpoint highlights the intricate terrain of cerebrovascular problems associated with SCD and the urgent need for all‐encompassing and fair strategies to improve patient outcomes and advance research.

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