TY  - CHAP
M1  - Book, Section
TI  - Thrombophilia in Sickle Cell Disease
A1  - M. Novelli, Enrico
A1  - Pawlinski, Rafal
A1  - Shiva, Sruti
A1  - Key, Nigel
A2  - Gladwin, Mark T.
A2  - Kato, Gregory J.
A2  - Novelli, Enrico M.
PY  - 2021
T2  - Sickle Cell Disease
AB  - Hemostatic  activation  is  a  hallmark  of  sickle  cell  disease  (SCD).  Cellular  activation  and  hyperadhesion,  sterile  inflammation,  hemolysis,  and  hemostatic  activation  contribute  to  a  chronic  coagulopathy  that  exacerbates  during  vaso-occlusive  episodes.  This  chapter  will  review  the  main  clinical  manifestations  of  thrombophilia  in  SCD  and  explore  its  pathogenic  mechanisms,  including  the  role  of  whole  blood  cellular  components  in  the  activation  of  the  intrinsic  and  extrinsic  coagulation  pathways,  the  main  alterations  of  procoagulant  and  anticoagulant  pathways  in  SCD,  and  the  role  of  hemolysis  and  nitric  oxide  depletion,  with  their  downstream  effects  on  endothelial  and  mitochondrial  dysfunction.  Finally,  the  role  of  established  and  novel  therapeutic  strategies  will  be  discussed.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/24
UR  - hemonc.mhmedical.com/content.aspx?aid=1179344908
ER  -