High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease

1991 ◽  
Vol 118 (3) ◽  
pp. 377-382 ◽  
Author(s):  
Winfred C. Wang ◽  
Edward H. Kovnar ◽  
Ina L. Tonkin ◽  
Raymond K. Mulhern ◽  
James W. Langston ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Recurrent Stroke ◽  
Cell Disease ◽  
Transfusion Therapy

Transcranial Doppler Measures In Patients with Sickle Cell Disease at High Risk for Stroke and Receiving Hydroxyurea: The HyRetro Ancillary Study.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1620-1620 ◽  
Author(s):  
Thomas Adamkiewicz ◽  
Nadine Odo ◽  
Abdullah Kutlar ◽  
Janet L Kwiatkowski ◽  
Robert J Adams
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Transfusion Therapy ◽  
Ancillary Study ◽  
Increased Risk

Abstract Abstract 1620 Children with sickle cell disease (SCD) and increased Transcranial Doppler (TCD) sonography velocity measures are at increased risk for stroke. Although chronic transfusion decreases this risk ten fold, this form of therapy is burdensome, and includes risk of iron overload. Although it has been established that the risk of stroke is still present even after 30 months of transfusion therapy, the total length of transfusion therapy required is not known. Hydroxyurea (HU) therapy is effective in preventing SCD complication, although its effect in preventing SCD central nervous system complications is less clear and a matter of current investigation. During one of the clinical trials (STOP2), an ancillary study was conducted to examine TCD velocities in 76 patients with SCD receiving hydroxyurea (HU) for a variety of indications. TCD measures were defined as the highest time-averaged maximum mean velocity of one of 8 measures (from the following cerebral arteries: LMCAVM, LM1VM, LBIFVM, LDICAVM, RMCAVM, RM1VM, RBIFVM and RDICAVM), as per STOP protocol. Results from 10 patients at high risk for stroke (defined as one or more TCD >=200 cm/sec) were evaluated for the present analysis. Average age at start of HU was 10.7 +/− 3.2 standard deviation (SD) years, 6 were female. Eight did not receiving chronic transfusion in STOP (randomized observation arm STOP n=4; observed non randomized in STOP n=4), and 2 were on transfusion (randomized transfusion arm STOP n=1; STOP/STOP2 with cross over n=1). Reasons for HU therapy included primary stroke prevention (n=6); secondary stroke prevention (TIA n=1; overt ischemic stroke n=1), vaso-occlusive or acute chest episodes (n=2). Averaged HU dose (available in 8) was 15 +/− 3 SD mg/kg. Averaged measures, off and on HU for each patient, were used to calculate means. Averaged hematological indices on treatment were as follows: white blood cell count 10.2 +/− 1.8 SD × 10^3/mm^3, hemoglobin 8.1 +/− 0.7 gm/dL; Mean Corpuscular Volume 105.6 +/− 6.7 cu μm; reticulocyte count 11.1 +/− 2.3 %. Averaged hemoglobin F, available in 8, was 16.1 +/− 5.7 %. Results are in table: Table Groups Treatment Months TCDs (n) cm/sec Not transfused in STOP (n=8) Pre HU 44+/−23 5.3+/−2.5 203+/−28 HU 33+/−24 5.0+/−3.6 179+/−48* Transfused in STOP (n=2) Pre HU 64+/−19 10.5+/−0.7 160+/−0.5 HU 19+/−7 4.5+/−0.7 180+/−1.1 * Wilcoxon signed-rank test p=0.008 One patient receiving HU for secondary stoke prevention suffered an overt stroke. This patient had a first overt stroke 24 months prior to start of HU therapy. MRI showed right frontal watershed and bilateral lacunar infarcts. Severe stenosis of the left MCA was noted. Patient had a repeat stroke 13 months after start of HU. MRI showed ischemia with watershed distribution of the left frontal lobe, stenosis of A2 and occlusion of A1 segments. Three TCD before and two after start of HU were all > 220 cm/sec. Overall, decreasing TCD velocities were noted in 60% prior to HU (STOP transfusion, n=2) and in 50% on HU (STOP transfusion, n=0). In conclusion, TCD velocities decreased significantly in high risk patients receiving HU, that were not transfused in STOP. However, these results require cautious interpretation, as numbers of patients are small, and length of observation varied. Patients with very high TCD measures remain at risk. Further studies may elucidate if there is a role for HU in patients with abnormal TCDs. Disclosures: No relevant conflicts of interest to declare.

Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke

2002 ◽  
Vol 140 (3) ◽  
pp. 348-354 ◽  
Author(s):  
Douglas J. Scothorn ◽  
Cynthia Price ◽  
Daniel Schwartz ◽  
Cindy Terrill ◽  
George R. Buchanan ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Recurrent Stroke ◽  
Cell Disease ◽  
Transfusion Therapy

scholarly journals Sickle Cell Anemia Presenting with Vaso-Occlusive Pain: Should We Screen for COVID-19?

2021 ◽  
pp. 1-4
Author(s):  
Mohammad Ali ◽  
Lina Okar ◽  
Nabil E. Omar ◽  
Jabeed Parengal ◽  
Ashraf Soliman ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Signs And Symptoms ◽  
Risk Groups ◽  
Position Statement ◽  
Interesting Case ◽  
International Federation ◽  
Cell Disease ◽  
The World

Despite the widespread of coronavirus disease-19 (CO­VID-19) infection around the world, there are very scarce reported literature about the care of patients with a known diagnosis of hemoglobin disorders such as sickle cell disease (SCD) or thalassemia and confirmed COVID-19 infection. Thalassemia International Federation issued a position statement to include patients with thalassemia and SCD among the high-risk groups of patients. Here, we present an interesting case of a 42-year-old patient know to have SCD presenting with Vaso-occlusive (VOC) pain episode in the absence of COVID-19 signs and symptoms, who tested positive for COVID-19 infection and had a smooth recovery. This case highlights the importance of screening SCD patients presenting with VOC-related events even in the absence of COVID-19 signs and symptoms.

Transfusion therapy for cerebrovascular abnormalities in sickle cell disease

1976 ◽  
Vol 88 (3) ◽  
pp. 382-387 ◽  
Author(s):  
Marie Olivieri Russell ◽  
Herbert I. Goldberg ◽  
Linda Reis ◽  
Shlomo Friedman ◽  
Robert Slater ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Transfusion Therapy

Abstract TP402: Sickle Cell Disease in Pediatric Stroke - Inpatient Resource Utilization in the Decade Following STOP

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
J. Michael Taylor ◽  
Paul Horn ◽  
Heidi Sucharew ◽  
Todd A Abruzzo ◽  
Jane Khoury
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Care Utilization ◽  
Cell Disease ◽  
Common Procedure ◽  
Transfusion Therapy ◽  
Stroke Population ◽  
Clinical Classification Software ◽  
Inpatient Database

Background: Sickle cell disease (SCD) is an important risk factor for stroke in children. Natural history studies demonstrate that greater than 10% of hemoglobin SS patients suffered ischemic stroke prior to age 20 years. In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) successfully demonstrated the role for routine transfusion therapy in reducing stroke in at risk SCD patients. Fullerton and colleagues then found that first time stroke in SCD decreased in Californian children in the 2 years following STOP. We investigated the stroke rate and health care utilization of children with SCD for two calendar years in the decade following publication of the STOP trial using a national inpatient database. Methods: The 2000 and 2009 Kids’ Inpatient Database (KID) were used for analysis. SCD and stroke cases were identified by ICD-9 codes 282.6x, 430, 431, 432.9, 434.X1, 434.9, 435.9. We queried the KID procedural clinical classification software for utilization of services pertinent to SCD and stroke; transfusion, MRI, and cerebral angio. Results: In 2000, SCD was a discharge diagnosis in 34,294 children and 158 (0.46%) children had SCD and stroke. By 2009, discharges with SCD rose to 37,082 children with 212 (0.57%) children carrying both diagnoses. In 2000 and 2009, AIS is the most common stroke type at 83%, males account for 53% of stroke and black race was reported by 92% of SCD and stroke subjects. Procedure utilization is higher in the SCD and stroke population than in SCD without stroke (Figure 1). Blood transfusion is the most common procedure in both study years, significantly higher in stroke subjects. Conclusion: For pediatric inpatients with SCD, blood transfusion and diagnostic cerebrovascular procedures were significantly more common in the cohort with comorbid stroke. In the decade after STOP, children hospitalized with SCD and stroke represented less than 0.6% of the total inpatient SCD population.

Retinal microvascular analysis using Fundus Fluorescein Angiography in Egyptian sickle cell disease patients

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Nayera H El Sherif ◽  
Mahmoud A Kenny ◽  
Waheed S Elhalfawy
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Fluorescein Angiography ◽  
Sickle Cell ◽  
Large Sample Size ◽  
Cell Disease ◽  
Fundus Fluorescein Angiography ◽  
Transfusion Therapy ◽  
Hydroxyurea Treatment ◽  
Trans Cranial Doppler

Abstract Background Sickle cell disease can affect retina of eye via vaso-occulsive changes that occur in micro-vessels of retina which could be analysed by using Fundus Fluorescein Angiography. Aim To analyze macular microvascular alternation in patients with SCD by Fundus Fluorescein Angiography (FFA) and to assess the role of potentially contributory Clinico-pathological factors including Trans-Cranial Doppler, genotypes, hydroxyurea, transfusion therapy and finally iron overload state on the development of macular alterations. Method This was across-sectional study which included 30 Sickle cell disease patients randomly recruited from the Paediatric Haematology clinic, children Hospital, Ain Shams University, Cairo, Egypt. Complete blood count (CBC), Trans-Cranial Doppler (TCD) and Fundus Fluorescein Angiography. Results In our study, there were 30 patients with mean age (14.1± 4.02), 5 patients had abnormal/conditional Trans-Cranial, 15 patients had Vaso-occlusive crises, 11 patients were on regular simple blood transfusion; all 30 studied sickle cell disease patients had normal Fundus Fluorescein Angiography and eye examination and only one patient hadabnormal visual acuity;A 29 years oldgirl who had five attacks of cerebral strokes last year, on regular simple blood transfusion and Hydroxyurea treatment with abnormal TCD and recurrent Vaso-occlusive crises in last two years, Although her vision is hand movement yet Fundus Fluorescein Angiography was normal. Conclusion we didn’t find any Retinal microvascular alternation in our studied SCD patients using Fundus Fluorescein Angiography, we related our results to the fact that our studied SCD patients were young and all our studied patients were on hydroxyurea therapy with fair compliance, further studies using large sample size are warranted in order to illustrate the utility of Fundus Fluorescein Angiography (FFA) as a tool for better detection of sickle retinopathy.

scholarly journals Ischemic Stroke in Children and Young Adults with Sickle Cell Disease (SCD) in the Post-STOP Era

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 68-68 ◽  
Author(s):  
Janet L. Kwiatkowski ◽  
Julie Kanter ◽  
Heather J. Fullerton ◽  
Jenifer Voeks ◽  
Ellen Debenham ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Clinical Series ◽  
The Mean

Abstract Background: The Stroke Prevention Trial in Sickle Cell Anemia (STOP) and Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) established routine transcranial Doppler ultrasound (TCD) screening with indefinite chronic red cell transfusions (CRCT) for children with abnormal TCD as standard of care. To identify children at high-risk of stroke, annual TCD screening is recommended from ages 2 to 16 years, with more frequent monitoring if the result is not normal. A reduction in stroke incidence in children with SCD has been reported in several clinical series and analyses utilizing large hospital databases when comparing rates before and after the publication of the STOP study in 1998. We sought to determine the rate of first ischemic stroke in a multicenter cohort of children who had previously participated in the STOP and/or STOP 2 trials and to determine whether these strokes were screening or treatment failures. Subjects and Methods: Between 1995 and 2005, STOP and STOP 2 (STOP/2) were conducted at 26 sites in the US and Canada. These studies included 3,835 children, ages 2 to 16 y with SCD type SS or S-beta-0-thalassemia. Participation in STOP/2 ranged from a single screening TCD to randomization. STOP 2 also had an observational arm for children on CRCT for abnormal TCD whose TCD had not reverted to normal. The Post-STOP study was designed to follow-up the outcomes of children who participated in one or both trials. 19 of the 26 original study sites participated in Post-STOP, contributing a total of 3,539 (92%) of the STOP/2 subjects. After exit from STOP/2, these children received TCD screening and treatment according to local practices. Data abstractors visited each clinical site and obtained retrospective data from STOP/2 study exit to 2012-2014 (depending on site) including follow-up TCD and brain imaging results, clinical information, and laboratory results. Two vascular neurologists, blinded to STOP/2 status and prior TCD and neuroimaging results, reviewed source records to confirm all ischemic strokes, defined as a symptomatic cerebral infarction; discordant opinions were resolved through discussion. For the first Post-STOP ischemic stroke, prior TCD result and treatment history subsequently were analyzed. Results: Of the 3,539 subjects, follow-up data were available for 2,850 (81%). Twelve children who had a stroke during STOP or STOP2 were excluded from these analyses resulting in data on 2,838 subjects. The mean age at the start of Post-STOP was 10.5 y and mean duration of follow-up after exiting STOP/2 was 9.1 y. A total of 69 first ischemic strokes occurred in the Post-STOP observation period (incidence 0.27 per 100 pt years). The mean age at time of stroke was 14.4±6.2 (median 13.8, range 3.5-28.9) y. Twenty-five of the 69 patients (36%) had documented abnormal TCD (STOP/2 or Post-STOP) prior to the stroke; 15 (60%) were receiving CRCT and 9 (36%) were not (treatment data not available for 1 subject). Among the 44 subjects without documented abnormal TCD, 29 (66%) had not had TCD re-screen in the Post-STOP period prior to the event; 7 of these 29 (24%) were 16 y or older at the start of Post-STOP, which is beyond the recommended screening age. Four of the 44 (9%) patients had inadequate TCD in Post-STOP (1 to 10.7 y prior to event). Six (14%) had normal TCD more than a year before the event (1.2 - 4 y); all but one of these children were younger than 16 y at the time of that TCD. Only 5 (11%) had a documented normal TCD less than 1 year prior to the event. Conclusions: In the Post-STOP era, the rate of first ischemic stroke was substantially lower than that reported in the Cooperative Study of Sickle Cell Disease, prior to implementation of TCD screening. Many (39%) of the Post-STOP ischemic strokes were associated with a failure to re-screen according to current guidelines, while only 11% occurred in children who had had recent low-risk TCD. Among those known to be at high risk prior to stroke, treatment refusal or inadequate treatment may have contributed. While TCD screening and treatment are effective at reducing ischemic stroke in clinical practice, significant gaps in screening and treatment, even at sites experienced in the STOP protocol, remain to be addressed. Closing these gaps should provide yet further reduction of ischemic stroke in SCD. Disclosures No relevant conflicts of interest to declare.

Sign in / Sign up

Export Citation Format

Share Document