Early Reticulocytosis and Anemia Are Associated with Abnormal and Conditional Transcranial Doppler Velocities in Children with Sickle Cell Anemia

Meier, Emily Riehm; Fasano, Ross M.; Estrada, Monica; He, Jie; Luban, Naomi L.C.; McCarter, Robert

doi:10.1016/j.jpeds.2015.10.031

Cited by 19 publications

(15 citation statements)

References 20 publications

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“…The patient's sex and Hb genotype did not influence the TAMMV in a consistent pattern. However, as reported previously, we also found that age, the Hb level, and the reticulocyte count showed significant correlations with the TAMMV. Although stenosis within an artery is probably the most substantial cause of increased velocity, the increased relative cardiac output in young children and in anemia could also affect the results.…”

Section: Discussionsupporting

confidence: 90%

Transcranial Doppler Ultrasound in Peninsular Arab Patients With Sickle Cell Disease

Adekile

Hassan

Asbeutah

et al. 2018

J of Ultrasound Medicine

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Section: Discussionsupporting

confidence: 90%

Transcranial Doppler Ultrasound in Peninsular Arab Patients With Sickle Cell Disease

Adekile

Hassan

Asbeutah

et al. 2018

J of Ultrasound Medicine

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“…Hence, we postulate that the presence of CD36 on the surface of immature sickle reticulocytes may contribute to the pathophysiology seen in SCA-associated vasculopathy, based on the associations observed between the higher ARC in patients with MRA-documented cerebrovasculopathy prior to initiating CTT which was unchanged after a year of CTT. This data suggest that this association is intrinsic to the patient regardless of CTT, and supports our previous data demonstrating that reticulocytosis is a hematologic marker of serious disease complications [ 39 ]. More detailed studies of reticulocyte adhesion properties in addition to the kinetics of their production post-transfusion are needed to better understand the biological relevance of ongoing or post-transfusional rebound reticulocytosis in these children.…”

Section: Discussionsupporting

confidence: 90%

Examination of Reticulocytosis among Chronically Transfused Children with Sickle Cell Anemia

et al. 2016

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Sickle cell anemia (SCA) is an inherited hemolytic anemia with compensatory reticulocytosis. Recent studies have shown that increased levels of reticulocytosis during infancy are associated with increased hospitalizations for SCA sequelae as well as cerebrovascular pathologies. In this study, absolute reticulocyte counts (ARC) measured prior to transfusion were analysed among a cohort of 29 pediatric SCA patients receiving chronic transfusion therapy (CTT) for primary and secondary stroke prevention. A cross-sectional flow cytometric analysis of the reticulocyte phenotype was also performed. Mean duration of CTT was 3.1 ± 2.6 years. Fifteen subjects with magnetic resonance angiography (MRA) -vasculopathy had significantly higher mean ARC prior to initiating CTT compared to 14 subjects without MRA-vasculopathy (427.6 ± 109.0 K/μl vs. 324.8 ± 109.2 K/μl, p<0.05). No significant differences in hemoglobin or percentage sickle hemoglobin (HbS) were noted between the two groups at baseline. Reticulocyte phenotyping further demonstrated that the percentages of circulating immature [CD36(+), CD71(+)] reticulocytes positively correlated with ARC in both groups. During the first year of CTT, neither group had significant reductions in ARC. Among this group of children with SCA, cerebrovasculopathy on MRA at initiation of CTT was associated with increased reticulocytosis, which was not reduced after 12 months of transfusions.

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“…Importantly, other previously reported biomarkers, notably the ARC , leucocyte counts , Lu‐BCAM expression and more generally adhesion molecules failed to provide prognostically significant insights in our study. Regarding reticulocyte and leucocyte counts, differences in study design might account for these discrepancies .…”

Section: Discussioncontrasting

confidence: 80%

Prognostic factors of disease severity in infants with sickle cell anemia: A comprehensive longitudinal cohort study

et al. 2018

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In order to identify very early prognostic factors that can provide insights into subsequent clinical complications, we performed a comprehensive longitudinal multi-center cohort study on 57 infants with sickle cell anemia (55 SS; 2 Sβ°) during the first 2 years of life (ClinicalTrials.gov: NCT01207037). Time to first occurrence of a severe clinical event-acute splenic sequestration (ASS), vaso-occlusive (VOC) event requiring hospitalization, transfusion requirement, conditional/ abnormal cerebral velocities, or death-was used as a composite endpoint. Infants were recruited at a mean age of 4.4 ±1 months. Median follow-up was 19.4 months. During the study period, 38.6% of infants experienced ≥1 severe event: 14% ASS, 22.8% ≥ 1 VOC (median age: 13.4 and 12.8 months, respectively) and 33.3% required transfusion. Of note, 77% of the cohort was hospitalized, with febrile illness being the leading cause for admission. Univariate analysis of various biomarkers measured at enrollment showed that fetal hemoglobin (HbF) was the strongest prognostic factor of subsequent severe outcome. Other biomarkers measured at enrolment including absolute neutrophil or reticulocyte counts, expression of erythroid adhesion markers, % of dense red cells, cellular deformability or ϒ-globin genetic variants, failed to be associated with severe clinical outcome. Multivariate analysis demonstrated that higher Hb concentration and HbF level are two independent protective factors (adjusted HRs (95% CI) 0.27 (0.11-0.73) and 0.16 (0.06-0.43), respectively). These findings imply that early measurement of HbF and Hb levels can identify infants at high risk for subsequent severe complications, who might maximally benefit from early disease modifying treatments.

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Early Reticulocytosis and Anemia Are Associated with Abnormal and Conditional Transcranial Doppler Velocities in Children with Sickle Cell Anemia

Cited by 19 publications

References 20 publications

Transcranial Doppler Ultrasound in Peninsular Arab Patients With Sickle Cell Disease

Transcranial Doppler Ultrasound in Peninsular Arab Patients With Sickle Cell Disease

Examination of Reticulocytosis among Chronically Transfused Children with Sickle Cell Anemia

Prognostic factors of disease severity in infants with sickle cell anemia: A comprehensive longitudinal cohort study

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