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Elevated circulating stromal-derived factor-1 levels in sickle cell disease.

P P Landburg¹, E Nur, N Maria

¹Immunology Laboratory Department, Red Cross Blood Bank Foundation Curaçao, Curaçao, Netherlands Antilles.

Acta Haematologica

|October 10, 2009

Summary

Serum levels of stromal-derived factor-1 (SDF-1) are elevated in sickle cell disease (SCD) patients, particularly those with HbSS/HbSbeta(0)-thalassaemia. These elevated SDF-1 levels may contribute to SCD-related pulmonary hypertension (PHT).

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Area of Science:

Hematology
Molecular Biology
Pathophysiology

Background:

Inflammation and angiogenesis are critical in sickle cell disease (SCD) pathophysiology.
Stromal-derived factor-1 (SDF-1) is a key mediator of angiogenesis and inflammation.

Purpose of the Study:

To determine serum SDF-1 levels in adult sickle cell patients.
To investigate the association between SDF-1 levels and clinical states (asymptomatic vs. painful crisis) and complications (pulmonary hypertension) in SCD.

Main Methods:

Serum samples were collected from adult SCD patients (HbSS/HbSbeta(0)-thalassaemia and HbSC/HbSbeta(+)-thalassaemia) and healthy controls.
SDF-1 levels were measured using immunoassays.
Patients were assessed during asymptomatic states and painful crises.
SDF-1 levels were compared between different SCD genotypes, clinical states, and presence/absence of pulmonary hypertension (PHT).

Main Results:

Serum SDF-1 levels were significantly elevated in HbSS/HbSbeta(0)-thalassaemia patients compared to HbSC/HbSbeta(+)-thalassaemia patients and healthy controls.
No significant increase in SDF-1 levels was observed during painful crises.
SDF-1 levels were significantly higher in SCD patients with pulmonary hypertension (PHT) than in those without PHT.

Conclusions:

Elevated circulating SDF-1 levels are present in patients with sickle cell disease.
SDF-1 may play a role in the pathophysiology of SCD, particularly in the development of SCD-related pulmonary hypertension.