Reversible posterior leuko‐encephalopathy in children with sickle cell disease

Khademian, Zarir; Speller‐Brown, Barbara; Nouraie, Mehdi; Minniti, Caterina P.

doi:10.1002/pbc.21812

Cited by 28 publications

(26 citation statements)

References 14 publications

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“…26 In a recent cohort of 80 children with SCD, who presented with acute neurological symptoms, 10% demonstrated appearances consistent with PRES on neuroimaging. 27 In this study, PRES was associated with systemic hypertension and increased mortality, but interestingly not with pre-existing brain infarcts or intracranial vasculopathy. PRES has also been reported following allogenous bone marrow transplantation (ABMT) to treat severe SCD, whereby myeloablative neurotoxicity has been postulated as a mechanism.…”

Section: Other Intracranial Abnormalities In Sickle Cell Diseasementioning

confidence: 47%

Neuroimaging findings in sickle cell disease

Thust

Burke²,

Siddiqui³

2014

BJR

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At least 25% of individuals with sickle cell disease will have a neurological complication over their lifetime, often as early as in childhood. Neuroradiological findings in patients with sickle cell disease are common and include acute territorial infarction, silent ischaemia and intracranial haemorrhage. Imaging abnormalities are typically, but not always, manifestations of the underlying vasculopathy. Coexisting acute and chronic pathology may pose challenges to interpretation.

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Section: Other Intracranial Abnormalities In Sickle Cell Diseasementioning

confidence: 47%

Neuroimaging findings in sickle cell disease

Thust

Burke²,

Siddiqui³

2014

BJR

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“…However, one-third of the patients developed PRES (a known complication of SCD and hemoglobinopathy transplants) that was reversible, despite our recommendation for strict blood pressure monitoring and prompt intervention. 53,54 Baseline blood pressure in SCD patients is generally lower than published norms for age, race, and sex, and the use of corticosteroid and calcineurin inhibitors may have exacerbated this complication. 53,[55][56][57] In conclusion, the trial met its prespecified 1-year EFS, and significantly improved HRQL was reported posttransplant.…”

Section: Discussionmentioning

confidence: 99%

A trial of unrelated donor marrow transplantation for children with severe sickle cell disease

Shenoy

Eapen

Panepinto

et al. 2016

Blood

171

157

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• Children with sickle cell disease engrafted unrelated donor marrow after reduced intensity conditioning.• A high incidence of GVHD and associated mortality compromised safety of the trial.Children with sickle cell disease experience organ damage, impaired quality of life, and premature mortality. Allogeneic bone marrow transplant from an HLA-matched sibling can halt disease progression but is limited by donor availability. A Blood and Marrow Transplant Clinical Trials Network (BMT CTN) phase 2 trial conducted from 2008 to 2014 enrolled 30 children aged 4 to 19 years; 29 were eligible for evaluation. The primary objective was 1-year event-free survival (EFS) after HLA allele-matched (at HLA-A, -B, -C, and -DRB1 loci) unrelated donor transplant. The conditioning regimen included alemtuzumab, fludarabine, and melphalan. Graft-versus-host disease (GVHD) prophylaxis included calcineurin inhibitor, short-course methotrexate, and methylprednisolone. Transplant indications included stroke (n 5 12), transcranial Doppler velocity >200 cm/s (n 5 2), ‡3 vaso-occlusive pain crises per year (n 5 12), or ‡2 acute chest syndrome episodes (n 5 4) in the 2 years preceding enrollment. Median follow-up was 26 months (range, 12-62 months); graft rejection was 10%. The 1-and 2-year EFS rates were 76% and 69%, respectively. The corresponding rates for overall survival were 86% and 79%. The day 100 incidence rate of grade II-IV acute GVHD was 28%, and the 1-year incidence rate of chronic GVHD was 62%; 38% classified as extensive. There were 7 GVHD-related deaths. A 34% incidence of posterior reversible encephalopathy syndrome was noted in the first 6 months. Although the 1-year EFS met the prespecified target of ‡75%, this regimen cannot be considered sufficiently safe for widespread adoption without modifications to achieve more effective GVHD prophylaxis.

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“…Although these features are different from classic stroke presentation, there has been at least one report of PRES mimicking focal stroke syndromes (Wernickes aphasia without hemiparesis) . There has been a surge in the literature describing this distinct neurological condition in patients with SCD …”

Section: Introductionmentioning

confidence: 99%

Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease

Solh

Taccone

Marin

et al. 2016

Pediatric Blood & Cancer

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Acute neurological changes in sickle cell disease (SCD) patients often raise the suspicion for stroke. Posterior reversible encephalopathy syndrome (PRES) can mimic stroke in its clinical presentation. We aimed to (i) review the PRES literature in SCD patients including clinical presentation, risk factors, pathophysiology, and management and (ii) elucidate the distinction between PRES and stroke in SCD. The exact pathophysiology of PRES in SCD remains elusive but is likely multifactorial and related to sickling, ischemia, and chronic anemia predisposing to vasogenic edema. PRES and stroke in SCD are distinguishable conditions. Our review may help elucidate a clinical approach to this distinction.

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Reversible posterior leuko‐encephalopathy in children with sickle cell disease

Cited by 28 publications

References 14 publications

Neuroimaging findings in sickle cell disease

Neuroimaging findings in sickle cell disease

A trial of unrelated donor marrow transplantation for children with severe sickle cell disease

Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease

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