Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2011 May;56(5):777-82.
doi: 10.1002/pbc.22951. Epub 2010 Dec 23.

Effect of transfusion therapy on transcranial Doppler ultrasonography velocities in children with sickle cell disease

Janet L Kwiatkowski  1 , Eunsil Yim, Scott Miller, Robert J Adams; STOP 2 Study Investigators
Collaborators, Affiliations

Effect of transfusion therapy on transcranial Doppler ultrasonography velocities in children with sickle cell disease

Janet L Kwiatkowski et al. Pediatr Blood Cancer. 2011 May.

Abstract

Background: Children with sickle cell disease (SCD) and abnormal transcranial Doppler (TCD) ultrasonography have a high risk of stroke, but this risk is greatly reduced when chronic transfusion therapy is administered. The change in TCD velocities during chronic transfusion therapy and rate and frequency of normalization of TCD findings have not been studied extensively.

Procedures: Using data from children with SCD enrolled as potential subjects in the Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) trial, we characterized the change in TCD velocities on transfusion therapy and identified predictors of developing a normal TCD.

Results: Among 88 children with serial TCD data after starting transfusions for abnormal TCD 46 (52%) converted to normal TCD after a mean of 4.3 months (median 3.0; range 0.85-14.3 months) of transfusions. TCD studies remained abnormal in 19/88 (21.6%) after a mean of 2.4 years of transfusion. The median TCD velocity was lowered by 38 cm/sec within 3 months of initiating transfusions, followed by a more gradual decline then stabilization of velocities, although with significant individual variation. Factors associated with conversion to normal TCD included lower initial TCD velocity, younger age, and higher pre-transfusion hemoglobin level during transfusion therapy.

Conclusion: Younger children with higher pre-transfusion hemoglobin levels and lower abnormal TCD velocities are most likely to have rapid normalization of TCD on transfusions. Long-term follow-up of children with persistently abnormal exams or worsening velocities on transfusion is needed to determine if these children are at higher risk of stroke.

PubMed Disclaimer

Conflict of interest statement

The authors state that there are no conflicts of interest relevant to this work.

Figures

Fig. 1
Fig. 1
(A) The change in TCD velocity following initiation of transfusion therapy for abnormal TCD is shown for all children (■), children who converted to normal TCD (▲) and children who never converted to normal TCD (□). Follow-up was censored at the time of randomization into the STOP 2 trial (17 subjects). (B) The change in TCD velocity over time from the prior value (three months before) is shown in the box and whisker plot (N = 88).
Fig. 2
Fig. 2
Kaplan Meier estimate of the proportion of children with abnormal TCD who converted to normal TCD while receiving transfusions is shown (N = 88).
Fig. 3
Fig. 3
Follow-up TCD results for the 88 children who began transfusion therapy for abnormal TCD during STOP 2 are shown.
See this image and copyright information in PMC

References

    1. Adams R, McKie V, Nichols F et al. The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med. 1992;326:605–610. - PubMed
    1. Adams RJ, McKie VC, Carl EM et al. Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Ann Neurol. 1997;42:699–704. - PubMed
    1. Adams RJ, McKie VC, Hsu L et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998;339:5–11. - PubMed
    1. Minniti CP, Gidvani VK, Bulas D et al. Transcranial Doppler changes in children with sickle cell disease on transfusion therapy. J Pediatr Hematol Oncol. 2004;26:626–630. - PubMed
    1. Bernaudin F, Verlhac S, Coic L et al. Long-term follow-up of pediatric sickle cell disease patients with abnormal high velocities on transcranial Doppler. Pediatr Radiol. 2005;35:242–248. - PubMed

Publication types