Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents

Wilimas, Judith A.; Goff, John R.; Anderson, Herbert; Langston, J. William; Thompson, Elizabeth I.

doi:10.1016/s0022-3476(80)80803-1

Cited by 159 publications

(58 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…We previously speculated 12 that the hyperemia observed in SCD plays a role in the occurrence of cerebrovascular events, particularly the ischemic events occurring in watershed areas of the brain. 5 The current data suggest that at similar Hb concentrations a high HbS fraction contributes to increase the hyperemia and possibly could increase the likelihood of cerebrovascular damage. The generalizability of this finding is limited not only by the small number of patients but also by the fact that all three already had strokes.…”

Section: Discussionmentioning

confidence: 59%

Effects of total hemoglobin and hemoglobin S concentration on cerebral blood flow during transfusion therapy to prevent stroke in sickle cell disease.

Hurlet‐Jensen

Prohovnik

Pavlakis

et al. 1994

Stroke

View full text Add to dashboard Cite

The standard treatment of stroke in sickle cell disease is chronic transfusion to maintain the fraction of abnormal hemoglobin (hemoglobin S [HbS]) below 20%. Risks associated with such transfusion can be reduced by allowing higher HbS levels, but the physiological consequences of this modification are unknown. Cerebral blood flow is elevated in sickle cell disease proportionate to the degree of anemia and is reduced by transfusion. We tested the effects of various HbS levels on cerebral blood flow during the course of transfusion therapy. We monitored cerebral blood flow (by the 133Xe inhalation method) in three patients whose chronic transfusion program was changed from a traditional regimen (HbS < 20%) to a moderate one, allowing HbS to rise to 45% to 50% between treatments. As expected, cerebral blood flow was higher with lower hemoglobin and higher HbS concentration. However, the HbS fraction appeared to exert a separate influence on the hyperemia, independent of total hemoglobin concentration. Furthermore, cerebral blood flow was higher during the modified regimen, despite equivalent anemia. These results suggest caution in adapting the modified transfusion regimen. Although HbS concentrations of 50% did not cause any frank neurological sequelae, the possible consequences of the associated hyperemia over time are unknown. We conclude that larger clinical and physiological studies of moderate transfusion regimens (allowing higher concentration of HbS) are necessary before it can become standard therapy.

show abstract

Section: Discussionmentioning

confidence: 59%

Effects of total hemoglobin and hemoglobin S concentration on cerebral blood flow during transfusion therapy to prevent stroke in sickle cell disease.

Hurlet‐Jensen

Prohovnik

Pavlakis

et al. 1994

Stroke

View full text Add to dashboard Cite

show abstract

“…746 In children with sickle cell anemia receiving no treatment, recurrence is as high as 92%. [747][748][749][750][751][752][753][754][755] Children with sickle cell anemia and transcranial Doppler (TCD) velocities . 200 cm/s have a 40% risk of stroke over the next 3 years.…”

Section: 58-259 Sickle Cell Anemiamentioning

confidence: 99%

Antithrombotic Therapy in Neonates and Children

Monagle

Chan

Goldenberg

et al. 2012

Chest

1,269

559

View full text Add to dashboard Cite

“…6 An effective therapeutic target for transfusions is 30% HbS, associated with a 14% to 23% incidence of secondary stroke and event rate of 2.2-6.4 recurrent strokes per 100 patient-years. 3,7,8 Unfortunately, chronic transfusions are administered indefinitely, because of the high stroke recurrence rate after discontinuation of short-term 9 or long-term 10 transfusions. Consequently, young patients with SCA and stroke remain on lifelong chronic transfusions with almost no available alternatives, and develop transfusion-associated problems including infections, erythrocyte auto/ alloimmunization, iron overload requiring chelation therapy, and reduced expected number of quality-adjusted life years.…”

Section: Introductionmentioning

confidence: 99%