RT Book, Section
A1 Schwinghammer, Terry L.
A1 DiPiro, Joseph T.
A1 Ellingrod, Vicki L.
A1 DiPiro, Cecily V.
SR Print(0)
ID 1178897020
T1 Sickle Cell Disease
T2 Pharmacotherapy Handbook, 11e
YR 2021
FD 2021
PB McGraw Hill
PP New York, NY
SN 9781260116694
LK accesspharmacy.mhmedical.com/content.aspx?aid=1178897020
RD 2022/08/18
AB Sickle  cell  syndromes,  which  can  be  divided  into  sickle  cell  trait  (SCT)  and  sickle  cell  disease  (SCD),  are  hereditary  conditions  characterized  by  the  presence  of  sickle  hemoglobin  (HbS)  in  red  blood  cells  (RBCs).SCT  is  the  heterozygous  inheritance  of  one  normal  β-globin  gene  producing  hemoglobin  A  (HbA)  and  one  sickle  gene  producing  HbS  (HbAS)  gene.  Individuals  with  SCT  are  asymptomatic.SCD  can  be  of  homozygous  or  compounded  heterozygous  inheritance.  Homozygous  HbS  (HbSS)  has  historically  been  referred  to  as  sickle  cell  anemia  (SCA),  which  now  also  includes  HbSβ0-thal  due  to  similarities  in  clinical  severity.  The  heterozygous  inheritance  of  HbS  with  another  qualitative  or  quantitative  β-globin  mutation  results  in  sickle  cell  hemoglobin  C  (HbSC),  sickle  cell  β-thalassemia  (HbSβ+-thal  and  HbSβ0-thal),  and  some  other  rare  phenotypes.