• Join Today!

Become a member and connect with:

  • An Active Online Community
  • Articles and Advice on SCD
  • Help Understanding Clinical Trials
abstracts & posters

Splenectomy to optimize hemoglobin s control in children with sickle cell disease on chronic transfusion therapy for stroke prevention

key information

source: The American Society of Pediatric Hematology/Oncology

year: 2016

authors: Jolie S. Ramesar, Catherine Boston, Vandy Black

summary/abstract:

Background: Consensus guidelines recommend chronic transfusion therapy (CTT) for stroke prevention in patients with sickle cell disease (SCD) who have a history of overt stroke or abnormal transcranial Doppler (TCD) velocities, typically with the goal of maintaining baseline hemoglobin (Hb) S <30%. Observational studies report considerable variation in Hb S% in children on CTT, but outside of alloimmunization, reasons for such variability have not been well-studied.

Objectives: We report the possibility of chronic splenic sequestration as a cause of poor Hb S control in 3 patients with SCD on CTT for stroke prevention and suggest consideration of splenectomy to optimize transfusion parameters in select high-risk children.

Design/Method: Patients were ages 7-9 years with Hb SS disease managed with chronic, partial-exchange PRBC transfusions every 3-4 weeks following a standardized institutional protocol with poor Hb S control. Indications for CTT were prior stroke and abnormal TCD. Surveillance magnetic resonance angiography showed progressive CNS vasculopathy. Spleens measured 9.5-13.5 cms. There was no evidence of alloimmunization. After careful consideration, these patients underwent laparoscopic splenectomy. Mean hematologic parameters were compared by paired t-tests 6 months pre and post splenectomy.

Results: Prior to splenectomy, mean pre-transfusion Hb was 7.4 gm/dL, reticulocyte count 20.7%, and Hb S 56%. Post splenectomy Hb was 9.5 gm/dL, reticulocyte count 3%, and Hb S 17%. Following splenectomy, there was a mean reduction in Hb S of 39.77% (95% CI 34.3-45.3, p<0.001). With a mean follow-up of 19 months, there were no perioperative or infectious complications. One patient appeared to have reduced iron burden with a mean reduction in baseline serum ferritin of 1,500 ng/mL, perhaps in part because his transfusions were spaced out due to improved Hb S control.

Conclusion: This data suggest splenic sequestration may cause shortened red cell survival resulting in suboptimal Hb S control and increased risk of incident or recurrent stroke. Splenectomy may be a reasonable therapeutic option for select high-risk patients. Additional research is warranted into the causes of variable Hb S control in patients with SCD on CTT so that therapeutic options can be refined to reduce the risk of overt stroke.

read more

To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More information

The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.

Close