• Join Today!

Become a member and connect with:

  • An Active Online Community
  • Articles and Advice on SCD
  • Help Understanding Clinical Trials
scientific articles

Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases

key information

source: Transfusion

year: 2015

authors: Santos B, Portugal R, Nogueira C, Loureiro M


Sickle cell anemia (SCA) is characterized by chronic hemolytic anemia. Worsening of anemia after red blood cell (RBC) transfusion has been reported and is often referred to as hyperhemolysis syndrome (HS). HS is a severe transfusion reaction characterized by destruction of both donor and host RBCs.
In this study we report the clinical findings and treatment of three adolescent patients with SCA who presented with HS. HS occurred after exchange transfusion in two cases and after RBC transfusion during vasoocclusive crises in one. A decrease of serum hemoglobin (Hb) levels was observed 5 to 7 days after transfusions and ranged from 2.5 to 5.7 g/dL. The direct antiglobulin test was positive in two patients who also presented with alloantibodies. Patient 3 received 2 additional RBC units after the onset of HS and experienced the lowest Hb values (2.5 g/dL) shortly after. Patients received intravenous steroids as the main specific treatment and also immunoglobulin and erythropoietin. Resolution of hemolysis was observed in all cases. Of note, spleen lesions were detected in two patients, one had a normal spleen size, and the other presented with enlarged spleen. Pathophysiologic implications of these findings were discussed.
HS must be a well-recognized complication of SCA after RBC transfusion. Prompt initiation of treatment and avoiding further transfusions may contribute to reduce the mortality associated with HS. The presence of functional spleen on the pathophysiology of HS deserves further evaluation.

organization: University Hospital Clementino Fraga Filho; Federal University of Rio de Janeiro

DOI: 10.1111/trf.12993

read more full text source

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.