RT Book, Section
A1 Li, Huihui
A1 S. Frenette, Paul
A2 Gladwin, Mark T.
A2 Kato, Gregory J.
A2 Novelli, Enrico M.
SR Print(0)
ID 1179338038
T1 Vaso-Occlusion in Sickle Cell Disease
T2 Sickle Cell Disease
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260458596
LK hemonc.mhmedical.com/content.aspx?aid=1179338038
RD 2024/04/18
AB Repetitive  vaso-occlusive  episodes  (VOEs)  are  the  most  common  clinical  manifestation  of  sickle  cell  disease  (SCD).  A  VOE  occurs  when  the  microcirculation  is  temporarily  or  permanently  obstructed  by  cellular  aggregates,  causing  ischemic  injury  to  the  supplied  organs  and  inducing  pain.  Acute  pain  and  chronic  organ  damage  provoked  by  the  vascular  occlusion  may  require  emergency  department  visits  or  hospitalization  for  affected  patients.  Clinical  presentations  induced  by  VOE  are  variable  and  summarized  in  Table  3-1.1,2  Although  acute  pain  is  a  major  reason  for  hospital  admission  and  permanent  organ  damage  is  a  frequent  cause  of  mortality  in  SCD  patients,3,4  whether  these  2  manifestations  are  linked  remains  unclear.  VOE-associated  ischemia  in  local  tissue  can  indeed  lead  to  organ  dysfunction,  but  the  etiology  of  these  injuries  is  very  complicated  and  not  yet  fully  understood.  For  instance,  the  primary  cause  of  acute  chest  syndrome  is  still  debated,  although  it  is  thought  to  be  a  combination  of  infection,  fat  embolism,  hypoventilation,  and  occlusion  of  the  pulmonary  vasculature.5  VOE  can  be  triggered  by  multiple  circumstances  such  as  dehydration,  infection  and/or  fever,  cold,  stress,  acidosis,  hypoxia,  and  pain  itself.4  In  addition,  environmental  factors,  such  as  geographic  altitude,  weather,  and  air  pollution,  are  likely  to  play  an  important  role  in  triggering  VOE.2,6  Changes  in  these  factors  may  promote  red  blood  cell  (RBC)  sickling  and  initiate  an  inflammatory  response  with  a  cascade  of  cellular  interactions  among  endothelial  cells,  leukocytes,  and  platelets  and  lead  to  vaso-occlusion  in  SCD  patients.  In  this  chapter,  we  will  review  the  pathophysiology  of  VOE  and  discuss  the  implications  for  the  development  of  suitable  biomarkers  of  disease  activity  and  therapeutic  targets  to  prevent,  treat,  or  cure  the  disease.