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ADAMTS13 activity in sickle cell disease
Author(s) -
Schnog JohnJohn B.,
Hovinga Johanna A. Kremer,
Krieg Soraya,
Akin Şakir,
Lämmle Bernhard,
Brandjes Dees P.M.,
Gillavry Melvin R. Mac,
Muskiet Fred D.,
Duits Ashley J.
Publication year - 2006
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20653
Subject(s) - medicine , disease , adamts13 , thrombotic thrombocytopenic purpura , platelet
Sickle red blood cell (SRBC)‐endothelial adhesion plays a central role in sickle cell disease (SCD)‐related vaso‐occlusion. As unusually large von Willebrand factor (ULVWF) multimers mediate SRBC‐endothelial adhesion, we investigated the activity of ADAMTS13, the metalloprotease responsible for cleaving ULVWF multimers, in SCD. ADAMTS13 activity was determined using a quantitative immunoblotting assay. VWF:Ag and VWF:RCo were determined using commercial assays. The high‐molecular‐weight VWF multimer percentage was determined by employing gel electrophoresis. ADAMTS13 activity was similar among asymptomatic patients ( n = 8), patients at presentation with a painful crisis ( n = 23), and healthy controls. ADAMTS13/VWF:Ag ratios were lower in patients compared to healthy HbAA controls, with the lowest values at presentation with a painful crisis ( P = 0.02). Division of samples in those with VWF:RCo/VWF:Ag ratios < 0.70 and those with ratios ≥ 0.70 revealed significantly more samples with ratios ≥ 0.70 ( P = 0.01) collected during painful crises. ULVWF multimers were detected in 6 patient samples and in 1 control sample. ADAMTS13/VWF:Ag ratios were inversely related to the duration of symptoms at presentation with an acute vaso‐occlusive event ( r s −0.67, P = 0.002). Although SCD is characterized by elevated VWF:Ag levels, no severe ADAMTS13 deficiency was detected in our patients. Am. J. Hematol. 81:492–498, 2006. © 2006 Wiley‐Liss, Inc.