Comparison of magnetic resonance angiography and conventional angiography in sickle cell disease: clinical significance and reliability

1996 ◽  
Vol 38 (5) ◽  
pp. 409-416 ◽  
Author(s):  
A. Y. Kandeel ◽  
R. A. Zimmerman ◽  
K. Ohene-Frempong
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Magnetic Resonance Angiography ◽  
Clinical Significance ◽  
Sickle Cell ◽  
Cell Disease ◽  
Conventional Angiography

Increased Pre-Transfusion Reticulocytosis Among Chronically Transfused Sickle Cell Disease Patients Is Associated With More Severe Cerebral Vasculopathy

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1163-1163
Author(s):  
Megha Kaushal ◽  
Ross M. Fasano ◽  
Colleen Byrnes ◽  
Naomi L.C. Luban ◽  
Emily Riehm Meier ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Magnetic Resonance Angiography ◽  
Sickle Cell ◽  
Control Group ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Blood Samples ◽  
History Of ◽  
The Mean

Abstract Chronic red blood cell transfusion therapy is indicated for primary and secondary stroke prevention in children with sickle cell disease (SCD). The main transfusion goal is achievement of pre-transfusion sickle hemoglobin (HbS) levels of 30%. Unfortunately, there continues to be a population of patients with a history of stroke who have progressive vasculopathy and/or secondary stroke, despite chronic transfusion therapy. Predictive markers for vasculopathy and cerebral events are needed to identify patients at risk for disease progression. Increased reticulocytosis was previously associated with other sickle cell disease complications, and adherent reticulocytes may contribute to the vascular pathology. The objective of this study was to explore the hypothesis that pre-transfusion reticulocytosis may serve as a disease severity marker for cerebral vasculopathy among chronically transfused children with sickle cell disease. After obtaining consent and assent, reticulocytosis was studied in a cohort of pediatric sickle cell patients treated with chronic transfusions (n=33, ages 2-17 years). The group was stratified into three groups: group 1 with an abnormal transcranial doppler (TCD) study in the absence of magnetic resonance angiography (MRA) detected vasculopathy [TCD(+), MRA(-), n=14], group 2 with a history of a stroke in the absence of MRA-detected vasculopathy [Stroke(+), MRA(-), n=5], and group 3 with a history of abnormal TCD or stroke and more severe vasculopathy detected by magnetic resonance angiography [MRA(+), n=14]. Pre-transfusion blood samples were analyzed within 72 hours of collection. Steady-state blood samples were also examined from a control group of pediatric SCD patients (>6 years of age) with normal TCD studies who were receiving supportive care in the absence of chronic transfusions or hydroxyurea (n=7). Hematologic data, including automated complete blood counts, absolute reticulocyte counts (ARC) with reticulocyte maturity were obtained. In addition, a flow cytometric approach was developed to further examine and quantitate reticulocyte subsets based upon staining with thiazole orange combined with CD36, CD45, CD49d, CD71, and CD235. The pre-transfusion HbS levels were not statistically different among the three transfused groups ([TCD(+), MRA(-)]: 30.2 ± 11.8%; [Stroke(+), MRA(-)]: 28.4 ± 3.3%; [MRA(+)]: 33.3 ± 9%, p>0.3). The high levels of reticulocytosis in the pre-transfusion samples were similar to those measured in the control group (ARC: 451 ± 126 K/uL in the chronically transfused cohort; ARC: 369 ± 94 K/uL in the control group, p=0.11). Pre-transfusion reticulocytosis was detected in every chronically transfused subject (ARC range 151-701 K/ul). The mean ARC in the [TCD(+), MRA(-)] group was not significantly different from the [Stroke(+), MRA(-)] group (411 ± 135 K/uL and 396 ± 97 K/uL respectively, p=0.82). However, the mean ARC in the [MRA(+)] group (512 ± 107 K/uL) was significantly higher than the control group, the [TCD(+), MRA(-)] group and the [Stroke(+), MRA(-)] group (p<0.05). The increased ARC in the MRA(+) group included higher absolute numbers of circulating reticulocytes at all stages of maturation including the immature reticulocyte subset [CD36(+), CD71(+)] that was detected in every sample. These data suggest that reticulocytosis with the release of immature CD36(+), CD71(+) cells into the peripheral blood remains as a characteristic feature of pediatric SCD even among chronically transfused patients. More severe vasculopathy, detected by MRA, was associated with significantly higher levels of pre-transfusion reticulocytosis. As such, reticulocytosis should be explored further as a marker or a potential contributor to more severe vasculopathy among chronically transfused children with SCD. Disclosures: No relevant conflicts of interest to declare.

WHY DO SICKLE SCREENING IN CHILDREN?

PEDIATRICS ◽  
1973 ◽  
Vol 51 (4) ◽  
pp. 742-745
Author(s):  
Norman Fost ◽  
Michael M. Kaback
Keyword(s):  
Genetic Counseling ◽  
Sickle Cell Disease ◽  
Clinical Significance ◽  
Sickle Cell ◽  
Early Identification ◽  
Cell Disease ◽  
Pilot Studies ◽  
Population Groups ◽  
Screening Programs

An approximate desire to "do something" about sickle cell disease has produced a plethora of sickle screening programs. While the advantages of early identification of individuals with sickle cell disease is apparent, the purpose of identifying children with sickle trait is less clear. There is uncertainty about the clinical significance of sickle trait, and there are ethical and legal hazards in being so identified. The demonstrated failure of genetic counseling to successfully transmit information in other population groups also dictates a reexamination of the presumed benefits of sickle screening programs. Screening programs should be initiated after pilot studies have resolved these issues, not before.

scholarly journals Transcranial Doppler and Magnetic Resonance in Tanzanian Children With Sickle Cell Disease

Stroke ◽  
2019 ◽  
Vol 50 (7) ◽  
pp. 1719-1726 ◽  
Author(s):  
Edward N. Kija ◽  
Dawn E. Saunders ◽  
Emmanuel Munubhi ◽  
Angela Darekar ◽  
Simon Barker ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Cell Disease

Clinical significance of circulating blood and endothelial cell microparticles in sickle-cell disease

2013 ◽  
Vol 38 (2) ◽  
pp. 167-175 ◽  
Author(s):  
Mutlu Kasar ◽  
Can Boğa ◽  
Mahmut Yeral ◽  
Suheyl Asma ◽  
Ilknur Kozanoglu ◽  
...  
Keyword(s):  
Endothelial Cell ◽  
Sickle Cell Disease ◽  
Clinical Significance ◽  
Sickle Cell ◽  
Cell Disease
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