RT Book, Section
A1 Castro, Oswaldo
A1 Hassell, Kathryn L.
A2 Gladwin, Mark T.
A2 Kato, Gregory J.
A2 Novelli, Enrico M.
SR Print(0)
ID 1179344817
T1 Rare Presentations and Emerging Complications of 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=1179344817
RD 2023/03/30
AB This  chapter  addresses  clinical  presentations  of  sickle  cell  disease  that  are  uncommon  or  newly  emerging  or  that  pose  special  diagnostic  and  therapeutic  challenges.  Many  of  them  are  life  threatening  or  can  result  in  severe  loss  of  organ  function.  Some  clinical  presentations  also  illustrate  features  of  sickle  cell  disease  pathophysiology  that  are  not  always  recognized  in  the  more  common  complications  such  as  pain  crisis  and  chest  syndrome.  There  are  no  clinical  trials  and  few  published  reviews  dealing  with  these  issues,  their  pathogenetic  mechanisms,  or  their  treatment.  Hence,  the  diagnostic  and  management  measures  suggested  here  necessarily  are  based  on  published  single  reports  and  small  case  series  and  on  clinical  experience.  Acute  multiorgan  failure  syndrome  is  summarized  first  because  of  its  life-threatening  potential  and  also  because  its  clinical  features  and  treatment  are  similar  to  some  of  the  other,  less  common  conditions  discussed.  Information  on  drug-induced  pain  episodes  (crises)  is  included  primarily  to  promote  awareness  of  these  newly  emerging  and  unexpected  complications.  The  chapter  ends  with  descriptions  of  how  other,  nonsickling,  red  cell  disorders  affect  patients  with  sickle  cell  disease,  with  a  special  emphasis  on  the  immune  hemolysis  that  characterizes  sickle-related  hyperhemolysis  syndrome.