Annals of Hematology

, Volume 93, Issue 7, pp 1139–1148

Elevated tricuspid regurgitant jet velocity in subgroups of thalassemia patients: insight into pathophysiology and the effect of splenectomy

  • Sylvia T. Singer
  • Frans Kuypers
  • Jeffery Fineman
  • Ginny Gildengorin
  • Sandra Larkin
  • Nancy Sweeters
  • Howard Rosenfeld
  • Gregory Kurio
  • Annie Higa
  • Michael Jeng
  • James Huang
  • Elliott P. Vichinsky
Original Article

DOI: 10.1007/s00277-014-2037-9

Cite this article as:
Singer, S.T., Kuypers, F., Fineman, J. et al. Ann Hematol (2014) 93: 1139. doi:10.1007/s00277-014-2037-9

Abstract

A high tricuspid regurgitant jet velocity (TRV) signifies a risk for or established pulmonary hypertension (PH), which is a serious complication in thalassemia patients. The underlying pathophysiology in thalassemia subgroups and potential biomarkers for early detection and monitoring are not well defined, in particular as they relate to spleen removal. To better understand some of these unresolved aspects, we examined 76 thalassemia patients (35 non-transfused), 25 splenectomized non-thalassemia patients (15 with hereditary spherocytosis), and 12 healthy controls. An elevated TRV (>2.5 m/s) was found in 25/76 (33 %) of the patients, confined to non-transfused or those with a late start of transfusions, including patients with hemoglobin H-constant spring, a finding not previously described. These non or late-transfused patients (76 % splenectomized) had significantly increased platelet activation (sCD40L), high platelet count, endothelial activation (endothelin-1), and hemolysis (LDH, plasma-free Hb), while hypercoagulable and inflammatory markers were not significantly increased. The same markers were increased in the seven patients with confirmed PH on cardiac catheterization, suggesting their possible role for screening patients at risk for PH. A combination of hemolysis and absence of spleen is necessary for developing a high TRV, as neither chronic hemolysis in the non-splenectomized thalassemia patients nor splenectomy without hemolysis, in the non-thalassemia patients, resulted in an increase in TRV.

Keywords

ThalassemiaSplenectomyHereditary spherocytosisPlatelet activationTRVPulmonary hypertension

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Sylvia T. Singer
    • 1
  • Frans Kuypers
    • 3
  • Jeffery Fineman
    • 7
  • Ginny Gildengorin
    • 4
  • Sandra Larkin
    • 3
  • Nancy Sweeters
    • 4
  • Howard Rosenfeld
    • 2
  • Gregory Kurio
    • 2
  • Annie Higa
    • 4
  • Michael Jeng
    • 6
  • James Huang
    • 5
  • Elliott P. Vichinsky
    • 1
  1. 1.Department of Hematology-OncologyChildren’s Hospital and Research Center OaklandOaklandUSA
  2. 2.Department of CardiologyChildren’s Hospital and Research Center OaklandOaklandUSA
  3. 3.Children’s Hospital Oakland Research InstituteOaklandUSA
  4. 4.Pediatric Clinical Research CenterChildren’s Hospital and Research Center OaklandOaklandUSA
  5. 5.Department of Hematology-OncologyUniversity of CaliforniaSan FranciscoUSA
  6. 6.Department of Hematology-OncologyStanford School of MedicinePalo AltoUSA
  7. 7.Department of PediatricsUniversity of California, San FranciscoSan FranciscoUSA