Published online 26 March 2008
Haematologica, Vol 93, Issue 5, 757-760 doi:10.3324/haematol.12152
Copyright © 2008 by Ferrata Storti Foundation
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Sickle Cell Disease

Sickle cell disease-related organ damage occurs irrespective of pain rate: implications for clinical practice

Eduard J. van Beers1, Charlotte F.J. van Tuijn1, Melvin R. Mac Gillavry2, Anna van der Giessen1, John-John B. Schnog2, Bart J. Biemond1, on behalf of the CURAMA study group

1 Department of Hematology, Academic Medical Center, Amsterdam
2 Department of Cardiology, Slotervaart Hospital, Amsterdam
3 Erasmus Medical Center, Rotterdam, The Netherlands

Correspondence: Bart J. Biemond, MD, Department of Haematology, F4-224, Academic Medical Center, PO box 22660, 1100 DD Amsterdam, The Netherlands. E-mail: b.j.biemond{at}amc.uva.nl

In daily clinical practice, the frequency of painful crises (pain rate) is an important parameter of sickle cell disease severity. We assessed the prevalence of sickle cell disease-related organ damage and complications and their relation to pain rate. Organ damage and history of vaso-occlusive complications were obtained via systematic screening of consecutive patients and by chart review. In 104 adult sickle cell patients pain rate was related to a history of acute chest syndromes, avascular osteonecrosis, iron overload, priapism and cholelithiasis. However, major disease-related complications, such as microalbuminuria and pulmonary hypertension, were detected in 23% and 24% respectively of patients without painful crises in the study period underlining the importance of systematic screening for developing organ damage in sickle cell patients irrespective of pain rate.

Key words: sickle cell disease, organ damage, pain rate, systematic screening.