TY - JOUR
T1 - Evolution of laboratory parameters during sickle cell painful crisis
T2 - Evidence compatible with dense red cell sequestration without thrombosis
AU - Billett, H. H.
AU - Nager, R. L.
AU - Fabry, M. E.
N1 - Funding Information:
From the Division of Hematology, Department of Medicine, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York. Supported by grants HL21016 and MM R01-050 from the National Institute of Health. The technical assistance of Kathleen Conroy and Joyce Johnson is gratefully acknowledged. The authors also thank E. Ezzone for her secretarial assistance. Reprint requests: HH Billett, MD, Ullman 903, AECOM, 1300 Morris Park Avenue, Bronx, NY 10461.
PY - 1988
Y1 - 1988
N2 - We find that during 51 episodes of sickle cell painful crisis indirect bilirubin fell 52% from its steady state value of 2.3 ± 1.9 mg% to a value of 1.1 ± 0.37 mg% at the end of crisis (p < .00000085). The indirect bilirubin decline correlates with a decrease in the dense sickle cells during crisis (r = .31, p < .0009). During steady state, both indirect bilirubin and lactic acid dehydrogenase correlate significantly with number of dense red cells (r = .62, p < .000002 and r = .32, p < .02 respectively). Platelet counts, β-thromboglobulin, Platelet Factor 4, and Fibrinopeptide A levels all were elevated during steady state and did not change during the evolution of crisis. These data demonstrate that elevated indices usually associated with platelet activation are a feature of the steady state of sickle cell disease but argue against thrombosis as a factor in the progression of a sickle cell painful crisis episode. The parallel decline of both dense cells and bilirubin during painful crisis indicates that the disappearance of dense cells during crisis is not caused by hemolysis and supports the hypothesis that dense red cell sequestration, in the absence of evidence of thrombosis, is an intrinsic component of the evolution of sickle cell painful crisis.
AB - We find that during 51 episodes of sickle cell painful crisis indirect bilirubin fell 52% from its steady state value of 2.3 ± 1.9 mg% to a value of 1.1 ± 0.37 mg% at the end of crisis (p < .00000085). The indirect bilirubin decline correlates with a decrease in the dense sickle cells during crisis (r = .31, p < .0009). During steady state, both indirect bilirubin and lactic acid dehydrogenase correlate significantly with number of dense red cells (r = .62, p < .000002 and r = .32, p < .02 respectively). Platelet counts, β-thromboglobulin, Platelet Factor 4, and Fibrinopeptide A levels all were elevated during steady state and did not change during the evolution of crisis. These data demonstrate that elevated indices usually associated with platelet activation are a feature of the steady state of sickle cell disease but argue against thrombosis as a factor in the progression of a sickle cell painful crisis episode. The parallel decline of both dense cells and bilirubin during painful crisis indicates that the disappearance of dense cells during crisis is not caused by hemolysis and supports the hypothesis that dense red cell sequestration, in the absence of evidence of thrombosis, is an intrinsic component of the evolution of sickle cell painful crisis.
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U2 - 10.1097/00000441-198811000-00001
DO - 10.1097/00000441-198811000-00001
M3 - Article
C2 - 2973751
AN - SCOPUS:0024273905
SN - 0002-9629
VL - 296
SP - 293
EP - 298
JO - American Journal of the Medical Sciences
JF - American Journal of the Medical Sciences
IS - 5
ER -