Original Paper

Elevated Circulating Stromal-Derived Factor-1 Levels in Sickle Cell Disease
P.P. Landburg^{a, b}, E. Nur^{c, d}, N. Maria^{a, b}, D.P.M. Brandjes^c, B.J. Biemond^d, J.B. Schnog^a-c, A.J. Duits^a, on behalf of the CURAMA Study Group

^aImmunology Laboratory Department, Red Cross Blood Bank Foundation Curaçao and
^bDepartment of Internal Medicine, St. Elisabeth Hospital, Curaçao, Netherlands Antilles;
^cDepartment of Internal Medicine, Slotervaart Hospital and
^dDepartment of Haematology, Academic Medical Center, Amsterdam, The Netherlands

Address of Corresponding Author

Acta Haematol 2009;122:64-69 (DOI: 10.1159/000243727)

  Key Words

• Sickle cell disease
• Stromal derived factor-1
• Pulmonary hypertension

  Abstract

Inflammation and angiogenesis are of importance in the pathophysiology of sickle cell disease (SCD). Recently, the chemokine stromal-derived factor-1 (SDF-1) has been shown to be a key mediator of angiogenesis and inflammation. In this study we determined serum SDF-1 levels in consecutive adult sickle cell patients during the clinically asymptomatic state as well as during painful crisis. Serum SDF-1 levels were significantly elevated in HbSS/HbS⁰-thalassaemia patients [n = 42; 5,177 pg/ml (2,438-7,246)] compared to HbSC/HbS⁺-thalassaemia patients [n = 16; 2,405 pg/ml (1,365-3,047)] and healthy HbAA controls [n = 45; 2,894 pg/ml (2,577-3,334)] (p = 0.001). No significant increments were observed during painful crisis (n = 40). SDF-1 levels were significantly higher in SCD patients with pulmonary hypertension (PHT) compared to patients without PHT. Elevated circulating SDF-1 levels occur in patients with SCD and may play a role in the pathophysiology of SCD-related PHT.

Copyright © 2009 S. Karger AG, Basel

  Author Contacts

Prof. Dr. A.J. Duits
Red Cross Blood Bank Foundation
Pater Euwensweg 36
Curaçao (Netherlands Antilles)
Tel. +599 9461 8433, Fax +599 9461 8431, E-Mail ajduits@gmail.com

  Article Information

Received: March 9, 2009
Accepted after revision: July 9, 2009
Published online: October 7, 2009
Number of Print Pages : 6
Number of Figures : 2, Number of Tables : 1, Number of References : 30