title: Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia.
abstract: OBJECTIVE
  The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD).
  
  
  METHODS
  The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy.
  
  
  RESULTS
  The analysis included 130 children, with 62.3% (n=81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4 g/dl, p<0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay.
  
  
  CONCLUSIONS
  Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS.
authors.affiliation: Duke University Medical Center, Durham, NC, United States.
authors.forename: Brian R
authors.initials: BR
authors.lastname: Englum
authors.affiliation: Duke University Medical Center, Durham, NC, United States.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Rothman
authors.affiliation: Duke University Medical Center, Durham, NC, United States.
authors.forename: Sarah
authors.initials: S
authors.lastname: Leonard
authors.affiliation: Duke University Medical Center, Durham, NC, United States.
authors.forename: Audra
authors.initials: A
authors.lastname: Reiter
authors.affiliation: Duke University Medical Center, Durham, NC, United States.
authors.forename: Courtney
authors.initials: C
authors.lastname: Thornburg
authors.affiliation: Calgary Children's Hospital, Calgary, AB, Canada.
authors.forename: Mary
authors.initials: M
authors.lastname: Brindle
authors.affiliation: Calgary Children's Hospital, Calgary, AB, Canada.
authors.forename: Nicola
authors.initials: N
authors.lastname: Wright
authors.affiliation: Boston Children's Hospital, Boston, MA, United States.
authors.forename: Matthew M
authors.initials: MM
authors.lastname: Heeney
authors.affiliation: Boston Children's Hospital, Boston, MA, United States.
authors.forename: C
authors.initials: C
authors.lastname: Jason Smithers
authors.affiliation: Cincinnati Children's Medical Center, Cincinnati, OH, United States.
authors.forename: Rebeccah L
authors.initials: RL
authors.lastname: Brown
authors.affiliation: Cincinnati Children's Medical Center, Cincinnati, OH, United States.
authors.forename: Theodosia
authors.initials: T
authors.lastname: Kalfa
authors.affiliation: Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Jacob C
authors.initials: JC
authors.lastname: Langer
authors.affiliation: Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Michaela
authors.initials: M
authors.lastname: Cada
authors.affiliation: Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: Keith T
authors.initials: KT
authors.lastname: Oldham
authors.affiliation: Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: J Paul
authors.initials: JP
authors.lastname: Scott
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Shawn D
authors.initials: SD
authors.lastname: St Peter
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Mukta
authors.initials: M
authors.lastname: Sharma
authors.affiliation: St. Jude Children's Research Hospital, Memphis, TN, United States.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Davidoff
authors.affiliation: St. Jude Children's Research Hospital, Memphis, TN, United States.
authors.forename: Kerri
authors.initials: K
authors.lastname: Nottage
authors.affiliation: St. Louis Children's Hospital, St. Louis, MO, United States.
authors.forename: Kathryn
authors.initials: K
authors.lastname: Bernabe
authors.affiliation: St. Louis Children's Hospital, St. Louis, MO, United States.
authors.forename: David B
authors.initials: DB
authors.lastname: Wilson
authors.affiliation: Stanford University, Palo Alto, CA, United States.
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Dutta
authors.affiliation: Stanford University, Palo Alto, CA, United States.
authors.forename: Bertil
authors.initials: B
authors.lastname: Glader
authors.affiliation: University of Arkansas, Little Rock, AR, United States.
authors.forename: Shelley E
authors.initials: SE
authors.lastname: Crary
authors.affiliation: University of Arkansas, Little Rock, AR, United States.
authors.forename: Melvin S
authors.initials: MS
authors.lastname: Dassinger
authors.affiliation: University of Florida, Gainesville, FL, United States.
authors.forename: Levette
authors.initials: L
authors.lastname: Dunbar
authors.affiliation: University of Florida, Gainesville, FL, United States.
authors.forename: Saleem
authors.initials: S
authors.lastname: Islam
authors.affiliation: University of Indiana, Indianapolis, IN, United States.
authors.forename: Manjusha
authors.initials: M
authors.lastname: Kumar
authors.affiliation: University of Indiana, Indianapolis, IN, United States.
authors.forename: Fred
authors.initials: F
authors.lastname: Rescorla
authors.affiliation: University of Michigan, Ann Arbor, MI, United States.
authors.forename: Steve
authors.initials: S
authors.lastname: Bruch
authors.affiliation: University of Michigan, Ann Arbor, MI, United States.
authors.forename: Andrew
authors.initials: A
authors.lastname: Campbell
authors.affiliation: University of Texas/MD Anderson Cancer Center, Houston, TX, United States.
authors.forename: Mary
authors.initials: M
authors.lastname: Austin
authors.affiliation: Emory University, Atlanta, GA, United States.
authors.forename: Robert
authors.initials: R
authors.lastname: Sidonio
authors.affiliation: Vanderbilt University Medical Center, Nashville, TN, United States.
authors.forename: Martin L
authors.initials: ML
authors.lastname: Blakely
authors.affiliation: Duke University Medical Center, Durham, NC, United States.
authors.forename: Henry E
authors.initials: HE
authors.lastname: Rice
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000601:surgery*; D001663:Bilirubin / Q000097:blood; D002648:Child; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D010535:Laparoscopy; D008297:Male; D012042:Registries; D017701:Reticulocyte Count; D013103:Spherocytosis, Hereditary / Q000097:blood* / Q000601:surgery*; D013156:Splenectomy / Q000379:methods*
chemical_list: D006454:Hemoglobins; D001663:Bilirubin
keywords: Congenital hemolytic anemia; Hematologic outcomes; Splenectomy; Surgical technique