An Elevated TRV Is Associated with Venous Thromboembolism in Sickle Cell Disease

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3222-3222
Author(s):  
Rachel M Adams ◽  
Lianne S Kopel ◽  
Demedrick Anton Bland ◽  
Elizabeth S Klings
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Medical Center ◽  
Heart Catheterization ◽  
Cell Disease ◽  
Complete Evaluation ◽  
Ct Angiogram ◽  
Significant Difference ◽  
History Of

Abstract Abstract 3222 Objective: An elevated tricuspid regurgitant jet velocity (TRV) by echocardiography, used to screen for pulmonary hypertension (PH) and mortality in sickle cell disease (SCD), likely has a multi-factorial etiology. SCD is a hyper-coagulable state, yet, the contribution of venous thromboembolic disease (VTE) to an elevated TRV is unknown. Based on the known association between VTE and PH, we hypothesized that SCD patients with an elevated TRV will have an increased prevalence of VTE. Methods: We reviewed data collected prospectively as part of the PH in SCD study conducted at Boston University/Boston Medical Center from 2004–2010. Subjects were included if they underwent both echocardiography and testing for VTE. An elevated TRV was defined as > 2.5 m/sec or the presence of PH on right heart catheterization. A history of VTE was defined by a positive CT/angiogram, ventilation/perfusion scan or Duplex ultrasound of an extremity (not catheter-related). Data were analyzed using a Chi-Square test and an odds ratio for VTE was calculated. Results: We reviewed the records of 162 patients enrolled in the PH of SCD study; 97 underwent both echocardiography and an evaluation for VTE.5/53 patients (9.4%) with normal echocardiography had a history of VTE, compared with 13/44 (29.5%) in the elevated TRV group. SCD patients with an elevated TRV were four times more likely to have a history of VTE compared to those with a normal echocardiogram (OR: 4.03, 95% CI 1.31–12.41, p=0.011). There was no significant difference in age, gender, history of asthma, hemoglobin genotype, hematologic profiles or renal function between patient groups. Conclusion: An elevated TRV and PH are associated with a history of VTE in SCD patients. This suggests a role for thrombosis in disease modulation and underscores the need for a complete evaluation for VTE in SCD patients with an elevated TRV. Disclosures: No relevant conflicts of interest to declare.

Five years of experience with hydroxyurea in children and young adults with sickle cell disease

Blood ◽  
2001 ◽  
Vol 97 (11) ◽  
pp. 3628-3632 ◽  
Author(s):  
Alina Ferster ◽  
Parvine Tahriri ◽  
Christiane Vermylen ◽  
Geneviève Sturbois ◽  
Francis Corazza ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Laboratory Data ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Significant Difference ◽  
History Of ◽  
Ischemic Attack

The short-term beneficial effect of hydroxyurea (HU) in sickle cell disease (SCD) has been proven by randomized studies in children and adults. The Belgian registry of HU-treated SCD patients was created to evaluate its long-term efficacy and toxicity. The median follow-up of the 93 patients registered is 3.5 years; clinical and laboratory data have been obtained for 82 patients at 1 year, 61 at 2 years, 44 at 3 years, 33 at 4 years, and 22 after 5 years. On HU, the number of hospitalizations and days hospitalized dropped significantly. Analysis of the 22 patients with a minimum of 5 years of follow-up confirm a significant difference in the number of hospitalizations (P = .0002) and days in the hospital (P < .01), throughout the treatment when compared to prior to HU therapy. The probabilities of not experiencing any event or any vaso-occlusive crisis requiring hospitalization during the 5 years of treatment were, respectively, 47% and 55%. On HU, the rate per 100 patient-years of severe events was estimated to be 3.5% for acute chest syndrome, 1.2% for aplastic crisis, 0.4% for splenic sequestration; it was 0% for the 9 patients with a history of stroke or transient ischemic attack followed for an average of 4 years. No important adverse effect occurred. Long-term chronic treatment with HU for patients with SCD appears feasible, effective, and devoid of any major toxicity; in patients with a history of stroke, HU may be a valid alternative to chronic transfusion support.

scholarly journals Sickle Cell Education for Medical Professionals: A Pre and Post Test Analysis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5812-5812
Author(s):  
Diana D Simmons ◽  
Robert Lucia ◽  
Kay Linn Saving ◽  
Nicole Alwan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Test Scores ◽  
Conflicts Of Interest ◽  
Learning System ◽  
Medical Professionals ◽  
Cell Disease ◽  
Learning Module ◽  
Significant Difference ◽  
Post Test

Abstract Background and Objectives: Sickle cell disease is a severe inherited form of anemia caused by a genetic mutation. Polymerization of hemoglobin leads to a cascade of effects decreasing blood flow. This causes tissue hypoxia leading to acute and chronic damage to the organs and endothelial lining. This disease requires complex management that relies on comprehensive training and knowledge regarding the disease process. Often accurate knowledge of sickle cell disease and how to provide appropriate care in the general medical population is limited. The purpose of this project was to develop a sickle cell educational training module for medical professionals. Such a module could be used to guide the provision of accurate education regarding sickle cell disease and best practice when caring for this patient population. Methods: Goals and learning objectives were created and current medical literature about caring for sickle cell disease was reviewed. A comprehensive PowerPoint presentation was produced along with a provider tip sheet and a pre and posttest. The presentation, tip sheet, and tests were reviewed by a board certified pediatric hematologist/oncologist along with the hospital's educational review committee in the Department of Professional Regulation. Once approved, the PowerPoint, tip sheet, and tests were combined into a learning module and uploaded onto an online learning system utilized by the hospital system. The module was sent to over 2,400 outpatient providers and staff and to all inpatient staff on units where sickle cell patients stay when admitted. The module consisted of the participant completing a 10 question pretest, then reviewing the PowerPoint presentation and tip sheet. Following the review of the PowerPoint and tip sheet, the participant completed a 10 question posttest and completed an evaluation of the module. Analysis: There were 223 people who completed the Sickle Cell Disease Learning Module. A paired t-test was conducted to compare pre-test scores to post-test scores. There was a significant difference in the pre-test scores (M = 5.98, SD = 1.66) and post-test scores (M = 9.17, SD = 1.36); p = <0.0001. Conclusion: The goal of this module was to increase baseline medical knowledge of sickle cell disease. The results indicate there was statistically significant improvement in baseline knowledge, based on pre and post data (p = <0.0001). While the results indicate statistically significant increases in performance, it would be important to see if improvements are sustained over time. Reassessment of participants one year after completion of module can be beneficial to see if learned knowledge has been retained. Disclosures No relevant conflicts of interest to declare.

A Multicentre Study of Environmental Factors on the Severity of Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4841-4841
Author(s):  
Sanjay Tewari ◽  
Fred Piel ◽  
Valentine Brousse ◽  
Baba PD Inusa ◽  
Paul Telfer ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Environmental Factors ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
Electronic Patient Records ◽  
Cell Disease ◽  
Hospital Data ◽  
Significant Difference

Abstract Background: Sickle cell disease (SCD) is a very variable condition, with some patients being asymptomatic and others admitted frequently to hospital. Genetic factors have been extensively investigated but only explain a small amount of the variability to date. Environmental factors are undoubtedly important, but have not been studied in depth, at least in part because of the difficulty of conducting these studies. We have analysed the role of climatic, environmental and temporal factors in determining the frequency of hospital admissions in children with SCD to 4 large sickle cell centres in London and Paris. Participants and Methods: Clinical data were collected from 1st January 2007 to 31st December 2012. Inclusion criteria were children with SCD (HbSS and HbSC) between the ages of 0 and 17 years, admitted to hospital with acute pain, acute chest syndrome or fever. All children lived within 4 miles radius (London) or 10km (Paris) of the hospital. Data were collected using specific electronic patient records of SCD patients. Data were collected on the reason for admission, date and length of admission. Daily air quality records were collected from sites around Paris and London, including details of black smoke, particulate matter, nitric oxide, carbon monoxide, sulphur dioxide and ozone. Daily meteorological records were obtained from weather stations in London and Paris including wind speed, temperature, rainfall and humidity. Statistical analysis including time series studies were conducted using R software version 3.1.1. Results: There were a total of 2717 admissions over the six year study period. Overall for the London hospitals there was a mean of 0.39 admissions/patient/year, with 1406 admissions for pain, 153 for acute chest syndrome and 417 for fever. The rate of admission/patient/year by cause for HbSS and HbSC across the London hospitals is shown in table below: Table 1. Rates of admission/patient/year by cause Sickle genotype/cause of admission All London hospitals Institution A Institution B Institution C HbSS (Pain) 0.31 0.18 0.40 0.43 HbSS (Fever) 0.09 0.03 0.15 0.11 HbSS (acute chest syndrome) 0.04 0.03 0.04 0.04 HbSC (pain) 0.07 0.03 0.08 0.10 HbSC (fever) 0.03 0.01 0.04 0.05 HbSC (acute chest syndrome) 0.004 0.008 0.002 0.002 Overall admission numbers were significantly higher on Mondays and Tuesdays in London but there was no such variation in Paris (Table 2). Table 2. Mean number of admissions on days of week in Paris (1 hospital) and London (3 hospitals). ** denotes significant difference from mean of other days (P<0.001). London Paris Weekday Monday 0.75** 0.35 Tuesday 0.77** 0.36 Wednesday 0.66 0.36 Thursday 0.64 0.32 Friday 0.60 0.32 Saturday 0.51 0.20 Sunday 0.57 0.27 There was no seasonal variation in admission numbers in London, but significantly higher numbers of patients admitted in Paris during autumn and winter. Table 3. Mean number of seasonal admissions in Paris (1 hospital) and London (3 hospitals). ** denotes significant difference from mean of other days (P<0.001). London Paris Season Autumn 0.70 0.35** Spring 0.60 0.31 Summer 0.64 0.25 Winter 0.62 0.34** Conclusion In London, there is a 2-3 fold variation in admission rates for the same complications between different hospitals. Similarly there is a significant difference on the effects of season and weekday between Paris and London. These results are statistically stronger than many effects which are identified in genetic and therapeutic studies, and show the importance of environmental and cultural factors, which are potentially modifiable. The effect of weather and pollution on hospital admissions is currently being analysed. Disclosures No relevant conflicts of interest to declare.

scholarly journals Hypoxia Enhanced Red Cell Adhesion in Vitro May Identify Patients at Risk for Vasculopathy

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3672-3672
Author(s):  
Charlotte Yuan ◽  
Erina Quinn ◽  
Sargam Kapoor ◽  
Myeongseop Kim ◽  
Erdem Kucukal ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Cell Disease ◽  
Hypoxic Conditions ◽  
Significant Difference ◽  
History Of ◽  
Male Subjects

Abstract Background: Priapism is a serious complication associated with Sickle Cell Disease (SCD) that may be a manifestation of underlying vasculopathy. The Centers for the Study of Complex Diseases of Childhood (CSCCD), comprising independent Comprehensive Sickle Cell Centers, demonstrated an association of priapism with hemolysis.1 Previously, we identified two groups of people with SCD based on red blood cell (RBC) adhesion under hypoxic conditions: those patients whose RBCs showed hypoxia-enhanced adhesion (HEA) and those whose did not (non-HEA).2 Patients with HEA had evidence for more hemolysis in vivo. Here, we aimed to examine (1) the association of HEA with hypoxia in vivo, and (2) RBC adhesion in normoxic and hypoxic conditions in male patients with or without a history of priapism. Methods: This retrospective study was conducted at the Adult Sickle Cell Disease Clinic at the University Hospitals Seidman Cancer Center, in Cleveland, OH between 2015 to 2018. Blood samples were obtained from 26 male subjects (29 samples, 25 HbSS and 1 HbSS HPFH). Adhesion experiments were performed as previously reported by passing surplus whole blood through LN-immobilized microchannels at physiological conditions under both normoxic and hypoxic conditions.2,3 Adherent RBCs were then quantified with microscope after a wash step. The median value was used for data analyses from multiple samples obtained from an individual. Chart review was conducted to examine results of hypoxia testing obtained in vivo as part of routine clinical care. Results: Male subjects with HbSS and a history of priapism had higher HEA in comparison to subjects without a history of priapism (3268 ± 5647 vs. 122 ± 1218, p=0.016). However, there was no significant difference between RBC adhesion of the two groups under normoxic conditions (529 ± 1528 vs. 402 ± 280). More male subjects with priapism had hypoxia in vivo (10 out of 14) than subjects without priapism (5 out of 12). Compared to male subjects with a history of priapism, those without a history of priapism had lower lactate dehydrogenase levels (474 ± 267 vs. 290 ± 215, p=0.008). Conclusions: Our data showed that subjects with a history of priapism had a higher HEA and tended to have more evidence for hypoxia in vivo than did subjects without a history of priapism. Further, male subjects with hypoxia in vivo had more HEA than did those without hypoxia in vivo (not shown). Hypoxia in vivo may cause increased RBC damage (reflected by HEA), hemolysis, nitric oxide depletion, and consequent vasculopathy, resulting in priapism. Hypoxia may be treatable, when identified in subjects with a history priapism in vivo or possibly with HEA in vitro. This could plausibly modify disease severity in some cases. References: Nolan VG, Wyszynski DF, Farrer LA, Steinberg MH. Blood. 20015 Nov;106(9):3264-7. doi: 10.1182/blood-2005-04-1594 Kim M, Alapan Y, Adhikari A, Little JA, Gurkan Microcirculation. 2017 Jul;24(5). doi: 10.1111/micc.12374. Alapan Y, Kim C, Adhikari A, Gray KE, Gurkan-Cavusoglu E, Little JA, Gurkan Transl Res. 2016 Jul;173:74-91.e8. doi: 10.1016/j.trsl.2016.03.008. Epub 2016 Mar 19. Disclosures Little: NHLBI: Research Funding; PCORI: Research Funding; Hemex: Patents & Royalties: Patent, no honoraria; Doris Duke Charitable Foundations: Research Funding.

Etiology of and Associations with Early Death in Adult Patients with Sickle Cell Disease At a Single Referral Institution.

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2117-2117
Author(s):  
Courtney Fitzhugh ◽  
Darlene Allen ◽  
Wynona Coles ◽  
Cassie Seamon ◽  
Xiongce Zhao ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Cause Of Death ◽  
Sickle Cell ◽  
Causes Of Death ◽  
Conflicts Of Interest ◽  
Organ Damage ◽  
Death Certificates ◽  
Cell Disease ◽  
Organ Function ◽  
Significant Difference

Abstract Abstract 2117 Sickle cell disease (SCD) causes significant morbidity and early mortality. The largest study to date reported in 1994 a median survival of 42 years for men and 48 years for women with homozygous SCD1. One third died during a vaso-occlusive crisis, and 18% died of acute organ failure. Circumstances of death were unknown in 18% of patients. With improved patient care in the current era including hydroxyurea (HU) therapy, we sought to identify age and causes of death and associated clinical variables in adults with SCD at a single referral institution. We first reviewed death certificates and assigned one or more causes of death based on all listed data. We studied autopsy reports and medical records and communicated with medical providers when available to identify further causes of death. We then performed a cross sectional analysis of clinical features obtained at initial enrollment and compared those variables in patients who are now living versus deceased using univariate t-test or Chi-squared analysis in order to determine which factors may be associated with death. 528 patients with SCD evaluated at the National Institutes of Health between 2001 and 2010 were included. Out of 511 patients with known genotypes, 391 patients had homozygous SCD. 85 of 528 (16%) died at a median age of 43 years for men and 44 years for women. Death certificates were available for 55 (65%) patients. SCD and infection were the most common listed cause of death (12% each), followed by pulmonary hypertension and/or cor pulmonale (9%), cardiac etiology (8%), narcotic toxicity (7%), and other (31%). Cause of death was unavailable in 18 (21%) cases. Deceased patients were significantly older at the time of first enrollment compared to living patients (41.5 vs. 34.1 years, p<0.0001). There was no significant gender difference between groups. There was also no significant difference in the reported use of HU or fetal hemoglobin levels. Enrollment laboratories suggesting renal insufficiency in deceased patients included a higher creatinine, phosphorus, and uric acid (p<0.02). Hepatic dysfunction was also more prevalent in the deceased group as evidenced by significantly higher direct bilirubin and alkaline phosphatase and lower albumin (p<0.005). There was no difference in alanine transaminase levels. Lactate dehydrogenase (LDH) was significantly higher in deceased patients (p=0.01). As there was no significant difference in hemoglobin, indirect bilirubin, or absolute reticulocyte counts between groups, higher LDH in deceased patients suggests a non-erythrocytic source. Ferritin levels and percent saturation of transferrin were significantly higher and transferrin significantly lower (p<0.004) suggesting more iron overload in deceased patients. Lastly, brain natriuretic peptide levels (reported only in patients with creatinine <1mg/dL) and tricuspid regurgitant velocity were also significantly increased (p<0.0001), suggesting higher prevalence of cardiopulmonary disease in deceased patients. In summary, the most common listed cause of death was nonspecific and unrevealing, reported as SCD. Surprisingly, another common cause of death was infection. This may be due to the combination of poor organ function reserve and functional asplenia. Deceased subjects were older and more likely to have organ impairment at initial evaluation. These data suggest that while contemporary management of patients with SCD may decrease acute manifestations, end organ damage still occurs. Lastly, markers of organ function should be closely monitored as patients increase in age, and organ-specific and definitive disease-specific therapy should be considered before irreversible organ damage ensues. 1 Platt OS et al. NEJM, 1994. 330(23): 1639–1644. Disclosures: No relevant conflicts of interest to declare.

The Effect Of Microalbuminuria On Duration Of Hospitalization In Adult Sickle Cell Disease Patients With Pain Crisis

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4694-4694
Author(s):  
Mohammed Shaik ◽  
Borys Hrinczenko
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Categorical Variables ◽  
Cell Disease ◽  
Exact Test ◽  
Blood Disorder ◽  
Significant Difference ◽  
Pain Crisis

Introduction Sickle cell disease (SCD) is a genetic blood disorder of hemoglobin resulting in severe morbidity and early mortality. The prevalence of microalbuminuria and/or proteinuria in children with SCD varies from 18 to 28% and increases with age. However, the exact prevalence in adults in not known. In the general population, the presence of albuminuria has been shown to be associated with all cause mortality. The urine microalbumin to creatinine ratio (MA) is considered to be an early sign of impending sickle cell nephropathy. We sought to investigate the association of MA with various SCD genotypes (HbSS, HbSC, HbS/β-thalassemia) in our clinic and also the length of stay (LOS) in hospitalized SCD patients with acute pain crisis. Methods Twenty-eight consecutive SCD patients diagnosed in our clinic by hemoglobin electrophoresis were included in our study. The patients (pts) age, gender, hemoglobin electrophoresis, and baseline MA were obtained. Based on their MA level they were divided into two groups, abnormal MA (MA ≥ 30 mg/g creatinine) or normal MA (< 30 mg/g creatinine). Eleven of these 28 patients were eventually hospitalized for a sickle cell related pain crisis. Their MA level was obtained within 24-hours of hospital admission and their hospitalization length of stay (LOS) was also recorded. We analyzed the association between the two groups of patients, those with normal or abnormal MA, with the different genetic variants of SCD in both our clinic and hospitalized pts. Furthermore, for hospitalized pts we also assessed an association of MA with their mean LOS. The Chi-square test/Fisher’s exact test was used for categorical variables and the T-test/Mann-Whitney test was used for numerical variables. Results All twenty-eight patients were African American without significant renal impairment, with 11, 10, and 7 pts with SS, SC and S β-thalassemia (Sβ), respectively. Fifteen of these pts had abnormal MA, 12 pts were female. The median age was 35.5 yrs (range, 19 - 59), median LOS was 3.5 days (range, 2-8). The SS pts had higher abnormal MA levels followed by SC and then Sβ (p=0.03) (Table 1). There was no significant difference in gender between the two groups (p=0.2). SCD pts admitted to the hospital for pain crisis with an abnormal MA within 24-hours of admission had a significantly higher mean LOS when compared to pts with normal MA (p=0.0089) (Table 1). Conclusion Microalbuminuria is more prevalent in the severe genotypes of SCD disease such as SS and SC vs. Sβ pts. Thereby, MA might be a useful biomarker of generalized SCD vasculopathy, in addition to a known marker of progressive nephropathy. Furthermore, MA has a significant impact on length of hospitalization. An abnormal MA obtained within the first 24-hrs of hospitalization of a SCD pt in pain crisis was predictive of prolonged hospital duration. Early and more aggressive supportive care symptom management for those patients might be a reasonable option but requires more study. Further large prospective clinical trials are needed to validate our findings. Disclosures: No relevant conflicts of interest to declare.

The Effect of Splenectomy on Development of Cerebral Vasculopathy in Children with Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3647-3647
Author(s):  
Azada Ibrahimova ◽  
Bruce Bernstein ◽  
Rida Abid ◽  
Nataly Apollonsky
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Sickle Cell Disease ◽  
Protective Effect ◽  
Sickle Cell ◽  
Cerebral Artery ◽  
Cell Disease ◽  
Increased Risk ◽  
Significant Difference ◽  
History Of

Abstract Splenic complications are often seen in pediatric patients with sickle cell disease and could lead to an increase in morbidity and mortality. The most common splenic event is acute splenic sequestration crisis (ASSC) which has been often managed with surgical splenectomy. Although splenectomy has been the treatment of choice for years, the long term complications of splenectomy on vascular events has not been broadly assessed. It has been reported that splenectomy could lead to vascular complications and increased risk of thrombosis in chronic hemolytic conditions. As it was proposed, it could be partially due to the shift form extravascular hemolysis to intravascular leading to the scavenging of nitric oxide (Kato et al, 2007). In terms of cerebral vasculopathy in children with sickle cell disease, screening is usually done by measuring blood flow velocity in the main cerebral arteries using Transcranial Doppler Ultrasonography (TCD). In 2014 Peter Soh and Abdul Siddiqui reported an increased risk of cerebrovascular complications in splenectomized patients with sickle cell disease (3), but it has not been confirmed by other publications. The aim of our study is to evaluate how splenectomy affects cerebral blood flow measured by TCD in patients with homozygous HbS. The secondary aim is to evaluate if Hydroxyurea (HU) has a protective effect on the development of cerebral vasculopathy in splenectomized patients. We performed a retrospective chart review of patients with sickle cell disease Hb SS and Hb S beta thalassemia followed at the Marian Anderson Center at St. Christopher's Hospital for Children, Philadelphia between 1999 and 2016. In the final analysis we included TCD data on 153 patients, of which 36 had undergone splenectomy. A total of 516 TCD studies were collected, of which 145 patients had a history of splenectomy. Of the 516 TCD assessments in this sample, 70 (13.6%) patients were on HU of which 21 had undergone splenectomy. Pearson correlations were calculated to explore linear associations of mean cerebral blood flow velocities (CBFV) with age at the time of TCD assessment, time since splenectomy, and TCD values. Associations of CVFV with splenectomy and with HU administration for splenectomy patients were analyzed with analysis of covariance (ANCOVA). The association of time elapsed since splenectomy to TCD measure, adjusting for age at TCD assessment, was explored with multiple linear regression models. Statistical analysis was conducted with SPSS ver. 24.0. Age at the time of TCD was significantly correlated with mean right cerebral artery (RMCA) (r = -.199, p < .001), LMCA (r = -.181, p < .001), RDICA (r = -.110, p = .047), LDICA (r = -.142, p = .01) and Basilar (r = -.186, p = < .001) velocities. Tables 1 and 2 present estimated mean TCD values (calculated adjusting for age at the time of TCD). In analysis of all TCDs, the estimated mean (RMCA) velocity was significantly higher in patients with history of splenectomy than in patients without splenectomy, (Table 1). There was also significant difference in LDICA, RDICA and right posterior cerebral artery (RPCA) velocities, all of which were higher in splenectomized patients (Table 1). When analyzing the velocities according to time since splenectomy, there were significant associations between years since splenectomy (0-5, 5-10 and >10years) and with both RMCA and LMCA abnormalities. Analysis of TCD results in splenectomized patients on HU versus non-HU, indicates all estimated mean TCDs except for RPCA, are lower for the patients on HU (Table 1), with RMCA and LMCA statistically significant. Based on our analysis we conclude that in patients with homozygous HbS disease history of surgical splenectomy increases the risk for cerebral vasculopathy. It remains unclear if cerebral vasculopathy develops as a result of splenectomy or if there is a preexisting factor leading to the development of both complications. The other important finding of our study is a protective effect of hydroxyurea on the development of cerebral vasculopathy in splenectomized patients. Disclosures No relevant conflicts of interest to declare.

Cognitive Dysfunction in Children and Adolescents with Sickle Cell Disease without Evidence of Stroke: Preliminary Results.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4623-4623
Author(s):  
Janete Silva ◽  
Ana Leite ◽  
Heber Maia ◽  
Ursula Thomé ◽  
Patricia Moura ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Sickle Cell Disease ◽  
Cerebrovascular Disease ◽  
Cognitive Dysfunction ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Cerebrovascular Disorders ◽  
Cell Disease ◽  
Neuropsychological Battery ◽  
Significant Difference

Abstract Abstract 4623 INTRODUCTION Sickle cell disease (SCD) is the most frequent cause of stroke in children. It is possible that cognitive dysfunction occurs in the absence of identifiable stroke due to silent cerebrovascular disorders. OBJECTIVE To evaluate SCD compromises cognitive functions of patients with no radiological nor clinical evidence of stroke. METHODS Thirty eutrophic children with SCD, with normal neurological examination and imaging (brain CT) and without past cerebrovascular disease were submitted to neuropsychological assessment between 2002 and 2008. The neuropsychological battery included the following tests: WISC-III, BTN (battery of neuropsychological tests, assessing laterality), Rey complex figure (visual perception and memory), and behavioral screening for ADHD, learning disorders and antisocial behavior. RESULTS 66.7% of patients were male. The average age of patients was 9.6 years (6 - 15 years). The mean total IQ was 78.8 (50 - 105) and the executive IQ was 77.9 (47 - 108), both at the borderline limit. The verbal IQ mean was 84.9 (55 - 113), on lower average. Verbal comprehension verbal was on average 87.4 (58 - 115). The results of visual memory was at the lower limit of normal (mean percentile 16 to 18). There was no correlation between the rates of behavior disorders (inattention, hyperactivity, learning difficulties and conduct disturbance) and IQ results. There was no statistically significant difference between the results of males and females or between age groups. 40% of the sample had not developed manual preference. CONCLUSION The subjects of this sample showed significant cognitive impairment in the absence of clinical or radiological evidence of cerebrovascular disease. The study has limitations as the sample size, the imaging technique used and neuropsychological battery comprehensiveness, but the results are quite suggestive of a relationship between SCD and cognitive impairment. The research group is developing a study with a larger sample (100 patients) to clarify the correlations between these findings. This will be important to outline earlier neuroprotective measures, which will provide improvements in neurodevelopment, learning, quality of life and social inclusion. Disclosures: No relevant conflicts of interest to declare.

Moyamoya Disease Predicts Progression of Cerebral Vasculopathy in Patients with Sickle Cell Disease Despite Chronic Transfusion Therapy

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2071-2071 ◽  
Author(s):  
Alyssa M Schlenz ◽  
Michael U Antonucci ◽  
Rebecca Cafiero ◽  
Nina-Serena Burkett ◽  
Julie Kanter
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Moyamoya Disease ◽  
Stroke Prevention ◽  
Conflicts Of Interest ◽  
The Body ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Moya Moya Disease ◽  
History Of

Abstract Introduction: Patients with sickle cell disease (SCD) develop multi-organ complications due to hemolysis, inflammation, and vascular occlusion that results in small vessel obstruction throughout the body. In the brain, however, large cerebral vessels are also damaged resulting in occlusion or stenosis and subsequent development of abnormal collateral vasculature and moyamoya disease. Chronic red cell transfusion (CRCT) therapy significantly reduces the risk of stroke in children with abnormal transcranial doppler (TCD) studies and is also effective in reducing stroke recurrence in those with a history of overt or silent stroke; however, it is unclear if CRCT halts or reverses the progression of vasculopathy. The present study evaluated cerebrovascular stenosis and moya moya disease as risk factors for progression of vasculopathy over time in a cohort of patients with SCD who were started on CRCT therapy as children for stroke prevention. Methods: A retrospective cohort study (with IRB approval) was used to evaluate cerebrovascular changes in patients on CRCT.Patients were included in the study if they had received CRCT for stroke prevention for at least 12 months and had at least two serial magnetic resonance imaging and angiography (MRI/MRA) studies for review. For the imaging analysis, the patient's MRI/MRA closest to the initiation of CRCT (i.e. baseline imaging) was compared to the most recent imaging available by a neuro radiologist who was blind to the patient's clinical history. Additional demographic information included the patient's current age, gender, indication for CRCT, years on CRCT, and laboratory results for pre-transfusion % hemoglobin S (HbS). Results: Forty patients with SCD (current age: M = 16.48, SD = 5.10; 23 male, 17 female) were included. Average duration of CRCT therapy was 9.96 years (SD = 5.67) and average pre-transfusion HbS levels were 42.52% (SD = 9.88). Patients were initiated on therapy due to: overt stroke (n = 19), silent stroke (n = 2), and abnormal TCD (n = 20). Of the 20 patients initiated on therapy due to abnormal TCD, 7 were found to have abnormal MRI at baseline consistent with silent stroke. One of these patients was also found to have co-occurring moyamoya disease, despite no evidence of prior overt stroke. At baseline, 45% (n = 18) of patients had abnormal MRA and 25% (n = 10) had moyamoya disease. Progression of vasculopathy occurred in 15% (n = 6) of patients, all of whom had a history of moya moya disease at baseline (5 patients with overt stroke and 1 with silent stroke). Of the remaining 3 patients with moya moya disease at baseline, 2 remained stable with no improvement and 1 demonstrated improvement on MRA. For patients with abnormal MRA, but no history of moya moya disease (n = 9), 5 demonstrated improvement (2 patients with silent stroke and 3 with overt stroke). Conclusions: Progression of vasculopathy was common among patients with baseline moyamoya disease despite CRCT. Also notable, however, was improvement in vasculopathy (as defined by reduction of stenosis) in 6 patients, the majority of whom had not developed moyamoya prior to the initiation of CRCT suggesting that more mild vasculopathy can be reversed with early intervention. Patients with moya moya disease warrant ongoing annual assessment as they may require vascular bypass to prevent further worsening. Future large, multi-site investigations are needed to identify improved biomarkers and further understand characteristics of patients who demonstrate improvement versus progression of vasculopathy on CRCT. Disclosures No relevant conflicts of interest to declare.

scholarly journals Frequency of Thrombotic Events in Hospitalized Patients with Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 13-13
Author(s):  
Susumu Inoue ◽  
Christina Anagonye
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Medical Center ◽  
Thrombotic Event ◽  
Beta Thalassemia ◽  
Control Group ◽  
Cell Disease ◽  
Very High Frequency ◽  
Thrombotic Risk

Background Thrombosis is one of the most common complications described in patients with sickle cell disease. However, little is known about the prevalence, variety and severity of thrombus particularly in sickle cell patients hospitalized with vaso-occlusive crisis. Methods We made a retrospective chart review of patients (age 21 years or older) admitted with sickle cell disease at Hurley Medical Center (Flint, Michigan) and was diagnosed with a thrombotic event between April 2012 and April 2020. To obtain a logistic model, we identified all patients with the the final discharge diagnosis of thrombosis (ICD-9 or ICD-10 code indicating any thrombosis, such as DVT, arterial thrombosis, catheter-related thrombus, pulmonary embolism, and stroke), combined with the diagnosis of sickle cell disease. Findings 37/137 (27%) sickle cell patients were found to have had at least one thrombotic event during their hospitalization. As predicted, patients with sickle cell disease appeared to have very high frequency of thrombotic complications, though we lack a control group to compare with. Genotypes of sickle cell disease was also measured in this study. Patients with the Hb-SS disease had a much higher rate of this complication(25/68=37%) compared with those with Hb SC disease patients (12/49=24%). There were no thrombotic events reported in patients with Hb S/beta thalassemia+, or 0 patients or in patients with Hb SS with HPFH. Interpretation The risk of a thromboembolic event in patients with SCD is high, Sickle cell anemia is a procoagulant condition, which is a risk factor for thrombosis. More research is required to determine if preventive modalities could reduce thrombotic risk. Disclosures No relevant conflicts of interest to declare.

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