ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso‐occlusive crisis of sickle cell disease

• Main Authors: Julien Demagny, Aurélie Driss, Alain Stepanian, Nadia Anguel, Louis Affo, Damien Roux, Anoosha Habibi, Sandrine Benghezal, Sophie Capdenat, Paul Coppo, Françoise Driss, Agnès Veyradier
• Format: Article
• Language: English
• Published: Wiley 2021-01-01
• Series: Research and Practice in Thrombosis and Haemostasis
• Subjects: ADAMTS13; sickle cell disease; thrombosis; vaso‐occlusion; von Willebrand factor
• Online Access: https://doi.org/10.1002/rth2.12460

Abstract Background Sickle cell disease (SCD) is characterized by vaso‐occlusive crisis (VOC), acute chest syndrome (ACS) and multiorgan failure (MOF) complicated by thrombosis. Von Willebrand factor (VWF) is a strong marker of SCD‐related endothelial injury. Objectives To decipher the role of VWF and its specific‐cleaving metalloprotease, ADAMTS13, in the vaso‐occlusive and thrombotic process of SCD. Patients/Methods We investigated the VWF antigen (Ag), ADAMTS13 activity, ADAMTS13 Ag and ADAMTS13 IgGs in a cohort of 65 patients with SCD prospectively enrolled in a 20‐month period from three centers. Patients were divided into two groups: an asymptomatic group (n = 30) with treated or untreated SCD at steady state, and a VOC/ACS group (n = 35) with SCD with VOC/ACS requiring either medical management or intensive care management for MOF. Results and Conclusions VWF:Ag levels were increased (median, 167 IU/dL; interquartile range [IQR], 124 ‐ 279), especially in patients with VOC SCD (227 IU/dL; IQR, 134‐305; P = .04), and positively correlated with inflammatory markers (P < .02). Median ADAMTS13 activity was normal (70 IU/dL; IQR, 60‐80), but 7 patients exhibited a partial deficiency between 25 and 45 IU/dL. ADAMTS13 activity/VWF:Ag ratio, however, did not change during VOC. Median ADAMTS13:Ag was slightly decreased (611 ng/mL; IQR, 504‐703) with no significant difference between groups. Surprisingly, ADAMTS13 IgGs were detected in 33 (51%) of our patients. We conclude that, in SCD, VWF:Ag and nonrelevant ADAMTS13 IgGs may reflect the severity of the inflammatory vasculopathy enhancing vaso‐occlusive and thrombotic complications.