Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.
The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.
Duke University Medical Center, Durham, North Carolina.
Henry E
HE
Rice
Brian R
BR
Englum
Jennifer
J
Rothman
Sarah
S
Leonard
Audra
A
Reiter
Courtney
C
Thornburg
Mary
M
Brindle
Nicola
N
Wright
Matthew M
MM
Heeney
Charles
C
Smithers
Rebeccah L
RL
Brown
Theodosia
T
Kalfa
Jacob C
JC
Langer
Michaela
M
Cada
Keith T
KT
Oldham
J Paul
JP
Scott
Shawn
S
St Peter
Mukta
M
Sharma
Andrew M
AM
Davidoff
Kerri
K
Nottage
Kathryn
K
Bernabe
David B
DB
Wilson
Sanjeev
S
Dutta
Bertil
B
Glader
Shelley E
SE
Crary
Melvin S
MS
Dassinger
Levette
L
Dunbar
Saleem
S
Islam
Manjusha
M
Kumar
Fred
F
Rescorla
Steve
S
Bruch
Andrew
A
Campbell
Mary
M
Austin
Robert
R
Sidonio
Martin L
ML
Blakely
D056586:Acute Chest Syndrome / Q000209:etiology / Q000473:pathology*; D000293:Adolescent; D000745:Anemia, Hemolytic, Congenital / Q000473:pathology / Q000601:surgery*; D000755:Anemia, Sickle Cell / Q000473:pathology / Q000601:surgery*; D017487:Ankyrins / Q000172:deficiency*; D001663:Bilirubin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000473:pathology*; D012042:Registries; D012141:Respiratory Tract Infections / Q000209:etiology / Q000473:pathology*; D012156:Reticulocytes / Q000473:pathology; D013103:Spherocytosis, Hereditary / Q000473:pathology / Q000601:surgery*; D013156:Splenectomy / Q000379:methods*; D016896:Treatment Outcome; D014481:United States
D017487:Ankyrins; D006454:Hemoglobins; D001663:Bilirubin