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

doi:10.4081/hr.2011.e12

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Case Report

1 September 2011

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

and

Clinic of Pediatric Haematology-Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, Via Giustiniani 2, 35100 Padova, Italy

Cardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, 35100 Padova, Italy

University of Padova, 35100 Padova, Italy

⁴

Bone Marrow Transplant Unit, Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, 35100 Padova, Italy

Hematol. Rep.2011, 3(2), e12;https://doi.org/10.4081/hr.2011.e12

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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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