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P-Selectin Blockade in the Treatment of Painful Vaso-Occlusive Crises in Sickle Cell Disease: A Spotlight on Crizanlizumab

Authors Karki NR, Kutlar A

Received 19 January 2021

Accepted for publication 13 March 2021

Published 30 March 2021 Volume 2021:14 Pages 849—856


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Robert B. Raffa

Nabin Raj Karki, Abdullah Kutlar

Division of Hematology/Oncology, Augusta University, Augusta, GA, USA

Correspondence: Nabin Raj Karki
Division of Hematology Oncology, Georgia Cancer Center at Augusta University, 1410, Laney Walker Blvd, CN 5347, Augusta, GA, 30912, USA
Tel +1-706-721-2505
Fax +1-706-721-5566
Email [email protected]

Abstract: Microvascular vaso-occlusion driven pain crisis is the hallmark of sickle cell disease with profound morbidity and increased mortality. Selectins, most notably P-selectins have an integral role in this phenomenon. P-selection was first identified in 1989. In 2019, after 3 decades of basic, translational, and clinical work with this pathway, the US Food and Drug Administration approved a P-selectin antibody, crizanlizumab to reduce frequency of pain crisis in patients more than 16 years with sickle cell disease. We review the fundamentals of P-selectin pathobiology, P-selectin blocking agents, clinical data with the use of crizanlizumab and prospects of this novel class of drugs in the context of other treatments for painful vaso-occlusive episodes.

Keywords: P-selectin, sickle cell disease, pain crisis, crizanlizumab

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