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Blood exchange transfusion safety for priapism in sickle cell disease: a single institution review

key information

source: The American Society of Pediatric Hematology/Oncology

year: 2016

authors: Maria O. Boucher, Patience Obasaju, Yara A. Park, Rupa Redding-Lallinger

summary/abstract:

Background: Males with sickle cell disease (SCD) have a 29-42% lifetime probability of developing priapism. Management of priapism is controversial and not evidence-based. Red cell exchange transfusion has been used to manage sickle related complications but several case reports have described acute neurological events following exchange transfusion for priapism, which limits enthusiasm for routine adoption of this therapy. Presently there are little data characterizing exchange transfusions utility and safety in treating priapism.

Objectives: To determine and compare the incidence of adverse events seen in patients with SCD receiving red cell exchange transfusion for refractory priapism and secondary stroke prophylaxis.

Design/Method: The University of North Carolina’s blood bank database was used to identify patients with SCD who were enrolled on chronic exchange transfusion program for refractory priapism from years 2004- 2015. An age-matched cohort of seven patients enrolled in exchange protocol for secondary stroke prophylaxis was identified.The first exchange procedure for both groups will be used for comparison. Adverse events such as vital sign instability and acute neurologic events were collected. Data collected will be analyzed to determine if there is a difference in adverse events in the priapism group compared to those receiving exchange transfusions for secondary stroke prophylaxis.

Results: We identified seven patients with SCD who were enrolled on a red cell exchange protocol for refractory priapism. We evaluated 6 episodes of exchange transfusions per patient. The mean age was 24; six patients have HbSS and one patient has HbSC. Two episodes of hypotension were recorded in one patient on two separate procedures.This resolved after decreasing the inlet pump flow rate to the patient. No adverse neurologic events were recorded. One patient experienced lip tingling that resolved with calcium carbonate tablets.The comparison data for the patients being transfused for secondary stroke prevention is being collected.

Conclusion: Although exchange transfusion is infrequently used for treating refractory priapism in patients with SCD, the incidence of adverse events appears to be minimal. Analysis is ongoing to compare the safety of red cell exchange transfusion for priapism to stroke.Future controlled trials using blood exchange transfusion for refractory priapism are needed.

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