Discontinuation of long-term transfusion therapy in patients with sickle cell disease and stroke

1997 ◽  
Vol 131 (5) ◽  
pp. 757-760 ◽  
Author(s):  
Sohail Rana ◽  
Patricia E. Houston ◽  
Narita Surana ◽  
Eglal I. Shalaby-Rana ◽  
Oswaldo L. Castro
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Transfusion Therapy

scholarly journals How I treat and manage strokes in sickle cell disease

Blood ◽  
2015 ◽  
Vol 125 (22) ◽  
pp. 3401-3410 ◽  
Author(s):  
Adetola A. Kassim ◽  
Najibah A. Galadanci ◽  
Sumit Pruthi ◽  
Michael R. DeBaun
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Management Strategies ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Hematopoietic Stem ◽  
Neuro Imaging

Abstract Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ∼11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by a multidisciplinary team (a hematologist, neurologist, neuroradiologist, and transfusion medicine specialist); prompt neuro-imaging and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion) is recommended if the hemoglobin is >4 gm/dL and <10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in SCD.

scholarly journals Strokes in children with sickle cell disease at the National Hospital Abuja Nigeria

2013 ◽  
Vol 40 (2) ◽  
pp. 158-164
Author(s):  
O Oniyangi ◽  
P Ahmed ◽  
OT Otuneye ◽  
J Okon ◽  
HA Aikhionbare ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Brain Imaging ◽  
Clinical Features ◽  
Sickle Cell ◽  
Stroke Recurrence ◽  
Cell Disease ◽  
Cerebral Infarcts ◽  
National Hospital ◽  
Transfusion Therapy

Background: Strokes occur in sickle cell disease (SCD), and are associated with significant morbidity and mortality.Objectives: To determine the prevalence of strokes amongst childrenwith SCD, and document the major clinical features, complications, effect of treatment with chronic transfusion therapy (CTT) and outcome.Methods: A descriptive retrospective study of SCD children with strokes seen at the National Hospital Abuja, Nigeria over a 2.5 year period from January 2009 – June 2012. Data was collected by scrutinizing case files obtained from the hospital medical records unit. Information obtained included demographic data, clinical features, packed cell volume (PCV), brain imaging, long term neurologic deficits, effect of CTT, stroke recurrence and outcome.Results: There were 31 children with strokes among 596 children with SCD documented in the register, giving a prevalence of 5.2%. Twenty six (26) case notes were retrieved. There were 12 males and 14 females, M: F ratio of 0.9:1; mean age was 6.4 years (SD 3.4) range: 1 year 7 months – 14 years; mean PCV at the time of strokes was 21.1% (SD 3.9) range 14 –29%. All (100%) had Haemoglobin SS on electrophoresis. Presentationswere convulsions 18, inability to use limbs 11, weakness of limbs 10; long term neurological deficits were hemiplegia 11, cognition loss 11. Three (3) children had no deficits. Brain imaging (Computed Tomography Scan and Magnetic Resonance Imaging) done in 16 (61.5%) children showed cerebral atrophy in 10, acute cerebral infarcts in 9, chronic cerebral infarcts in 6, acute intra cranial haemorrhage in 1 and normal imagings in 4 children. Twelve (12) children (46.2%) children had recurrences of stroke ranging in number from 1 to 4, which occurred 6 months to 3 years afterthe initial stroke. There were no statistical significant differences between the children with recurrences of stroke compared to those without regarding the age, sex, weight or PCVs p > 0.05. Fifteen (15) children (57.7%) wereenrolled in CTT. Two (2) out of 7 children (28.6%) that had regular CTT had stroke recurrence; compared to 5 out of 11 children (45.4%) with no CTT (p > 0.05). Four (4) out of 6 (66.7%) children with irregular CTT and 1 of 2 children who stopped CTT had stroke recurrence.Outcome: 17 children were alive, 7 were lost to follow up, 1 died and 1 was referred to another center.Conclusion: Strokes were an important cause of morbidity in Nigerianchildren with SCD, with major long term neurologic deficits. CTT appearedbeneficial in preventing stroke recurrences. Primary prevention strategy by Trans Cranial Doppler ultrasound studies of the cerebral arteries, with the aim of promptly initiating appropriate preventive therapy for stroke is strongly advocated.Key words: Sickle cell disease, Stroke, Children, Chronic Transfusion Therapy

scholarly journals Hypertransfusion Therapy in Sickle Cell Disease in Nigeria

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Ademola Samson Adewoyin ◽  
Jude Chike Obieche
Keyword(s):  
Health Status ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Component ◽  
Transfusion Medicine ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Careful Patient ◽  
Prevention And Treatment

Introduction. Hypertransfusion refers to chronic blood transfusion therapy aimed at ameliorating disease complications in various haemopathies particularly the haemoglobinopathies. In sickle cell disease, hypertransfusion is aimed at maintaining patient’s haemoglobin level at 10 to 11 g/dL using haemoglobin AA blood and its resultant dilutional effect on sickle haemoglobin is sustained by intermittent long-term transfusions.Aim and Objective. This paper highlights hypertransfusion and its privileged position as a secondary measure in prevention and treatment of sickle cell disease, especially in the Nigerian context.Materials and Methods. Relevant literatures were searched on PubMed, Google Scholar and standard texts in haematology and transfusion medicine. Keywords used in the search are hypertransfusion, sickle cell disease, chronic transfusion, and Nigeria. Literatures gathered were reviewed, summarized, and presented in this paper.Result. Immense clinical benefit is associated with hypertransfusion therapy including prevention of stroke and amelioration of severe sickle cell disease especially in transplant ineligible patients. Careful patient selections, appropriate blood component, and prevention of transfusion hazards as well as oversight function of an experienced haematologist are pertinent to a successful hypertransfusion therapy.Conclusion. Improved knowledge of the benefits and practice of hypertransfusion will effectively translate into improved health status even among Nigerian sickle cell disease patients.

Increased blood requirements during long-term transfusion therapy for sickle cell disease

1991 ◽  
Vol 118 (3) ◽  
pp. 405-407 ◽  
Author(s):  
Alan R. Cohen ◽  
George R. Buchanan ◽  
Marie Martin ◽  
Kwaku Ohene-Frempong
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Transfusion Therapy

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.

Returns on long-term investments in sickle cell disease

Blood ◽  
2004 ◽  
Vol 103 (11) ◽  
pp. 3996-3996
Author(s):  
Alan R. Cohen
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease
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