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Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation

Raffaella Colombatti, Elena Varotto, Silvia Ricato, Daniel Nardo, Nicola Maschietto, Simone Teso, Marta Pillon, Chiara Messina, Ornella Milanesi, Laura Sainati

Authors information
  • Raffaella Colombatti
    Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy. rcolombatti@gmail.com
  • Elena Varotto
    Cardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.
  • Silvia Ricato
    Cardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.
  • Daniel Nardo
    Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.
  • Nicola Maschietto
    Cardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.
  • Simone Teso
    University of Padova, Padova, Italy.
  • Marta Pillon
    Bone Marrow Transplant Unit, Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.
  • Chiara Messina
    Bone Marrow Transplant Unit, Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.
  • Ornella Milanesi
    Cardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.
  • Laura Sainati
    Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Padova, Italy.

Abstract


Elevated Tricuspid Regurgitant Velocity (TRV) has been related to higher mortality in adults and to hemolysis, lower oxygen saturation during 6-minute walk test and acute chest syndrome (ACS) in children with sickle cell disease (SCD). Hydroxyurea (HU) has reduced TRV value in children and adults. We describe a three year old HbSS child with recurrent ACS, hypoperfusion of the left lung, mild hemolysis and persistent TRV elevation. TRV did not normalize after HU, despite improvement in clinical conditions and in baseline laboratory parameters related to hemolysis and blood viscosity, but normalized after bone marrow transplantation (BMT). Our experience suggests that in young patients, TRV reduction can be a positive concomitant effect of BMT.

Keywords


sickle-cell disease, pulmonary hypertension, tricuspid regurgitant jet velocity, hydroxyurea, bone marrow transplantation.

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Submitted: 2011-05-26 14:24:46
Published: 2011-09-01 10:23:10
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Hematology Reports [eISSN 2038-8330] is an Open Access, peer-reviewed journal published by PAGEPress, Pavia, Italy. All credits and honors to PKP for their OJS.

 
 
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