• In children with AIC, IMs are associated with a lower splenectomy FFS.

  • IMs are associated with a higher risk of recurrent or severe bacterial infections and thrombosis.

Splenectomy is effective in ∼70% to 80% of pediatric chronic immune thrombocytopenia (cITP) cases, and few data exist about it in autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES). Because of the irreversibility of the procedure and the lack of predictions regarding long-term outcomes, the decision to undertake splenectomy is difficult in children. We report here factors associated with splenectomy outcomes from the OBS’CEREVANCE cohort, which prospectively includes French children with autoimmune cytopenia (AIC) since 2004. The primary outcome was failure-free survival (FFS), defined as the time from splenectomy to the initiation of a second-line treatment (other than steroids and intravenous immunoglobulins) or death. We included 161 patients (cITP, n = 120; AIHA, n = 19; ES, n = 22) with a median (minimum-maximum) follow-up of 6.8 years (1.0-33.3) after splenectomy. AIC subtype was not associated with FFS. We found that immunopathological manifestations (IMs) were strongly associated with unfavorable outcomes. Diagnosis of an IM before splenectomy was associated with a lower FFS (hazard ratio [HR], 0.39; 95% confidence interval [CI], 0.21-0.72, P = .003, adjusted for AIC subtype). Diagnosis of an IM at any timepoint during follow-up was associated with an even lower FFS (HR, 0.22; 95% CI, 0.12-0.39; P = 2.8 × 10−7, adjusted for AIC subtype) as well as with higher risk of recurrent or severe bacterial infections and thrombosis. In conclusion, our results support the search for associated IMs when considering a splenectomy to refine the risk-benefit ratio. After the procedure, monitoring IMs helps to identify patients with higher risk of unfavorable outcomes.

1.
Pincez
T
,
Fernandes
H
,
Leblanc
T
, et al
.
Long term follow-up of pediatric-onset Evans syndrome: broad immunopathological manifestations and high treatment burden
.
Haematologica.
2022
;
107
(
2
):
457
-
466
.
2.
Hadjadj
J
,
Aladjidi
N
,
Fernandes
H
, et al;
members of the French Reference Center for Pediatric Autoimmune Cytopenia (CEREVANCE)
.
Pediatric Evans syndrome is associated with a high frequency of potentially damaging variants in immune genes
.
Blood.
2019
;
134
(
1
):
9
-
21
.
3.
Teachey
DT
,
Lambert
MP
.
Diagnosis and management of autoimmune cytopenias in childhood
.
Pediatr Clin North Am.
2013
;
60
(
6
):
1489
-
1511
.
4.
Moulis
G
,
Palmaro
A
,
Montastruc
J-L
,
Godeau
B
,
Lapeyre-Mestre
M
,
Sailler
L
.
Epidemiology of incident immune thrombocytopenia: a nationwide population-based study in France
.
Blood.
2014
;
124
(
22
):
3308
-
3315
.
5.
Ducassou
S
,
Gourdonneau
A
,
Fernandes
H
, et al;
Centre de Référence National des Cytopénies Auto-immunes de l’Enfant (CEREVANCE)
.
Second-line treatment trends and long-term outcomes of 392 children with chronic immune thrombocytopenic purpura: the French experience over the past 25 years
.
Br J Haematol.
2020
;
189
(
5
):
931
-
942
.
6.
Chaturvedi
S
,
Arnold
DM
,
McCrae
KR
.
Splenectomy for immune thrombocytopenia: down but not out
.
Blood.
2018
;
131
(
11
):
1172
-
1182
.
7.
Mageau
A
,
Terriou
L
,
Ebbo
M
, et al
.
Splenectomy for primary immune thrombocytopenia revisited in the era of thrombopoietin receptor agonists: New insights for an old treatment
.
Am J Hematol.
2022
;
97
(
1
):
10
-
17
.
8.
Neunert
C
,
Terrell
DR
,
Arnold
DM
, et al
.
American Society of Hematology 2019 guidelines for immune thrombocytopenia
.
Blood Adv.
2019
;
3
(
23
):
3829
-
3866
.
9.
Mannering
N
,
Hansen
DL
,
Frederiksen
H
.
Evans syndrome in children below 13 years of age - a nationwide population-based cohort study
.
PLoS One.
2020
;
15
(
4
):
e0231284
.
10.
Mathew
P
,
Chen
G
,
Wang
W
.
Evans syndrome: results of a national survey
.
J Pediatr Hematol Oncol.
1997
;
19
(
5
):
433
-
437
.
11.
Mantadakis
E
,
Farmaki
E
.
Natural history, pathogenesis, and treatment of Evans syndrome in children
.
J Pediatr Hematol Oncol.
2017
;
39
(
6
):
413
-
419
.
12.
Sokol
RJ
,
Hewitt
S
,
Stamps
BK
,
Hitchen
PA
.
Autoimmune haemolysis in childhood and adolescence
.
Acta Haematol.
1984
;
72
(
4
):
245
-
257
.
13.
Ducassou
S
,
Fernandes
H
,
Savel
H
, et al
.
Prospective evaluation of the first option, second-line therapy in childhood chronic immune thrombocytopenia: splenectomy or immunomodulation
.
J Pediatr.
2021
;
231
:
223
-
230
.
14.
Aronis
S
,
Platokouki
H
,
Avgeri
M
,
Pergantou
H
,
Keramidas
D
.
Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children
.
Acta Paediatr.
2004
;
93
(
5
):
638
-
642
.
15.
Avila
ML
,
Amiri
N
,
Pullenayegum
E
,
Blanchette
V
,
Imbach
P
,
Kühne
T
;
Intercontinental Cooperative Itp Study Group
.
Long-term outcomes after splenectomy in children with immune thrombocytopenia: an update on the registry data from the Intercontinental Cooperative ITP Study Group
.
Haematologica.
2020
;
105
(
11
):
2682
-
2685
.
16.
Kojouri
K
,
Vesely
SK
,
Terrell
DR
,
George
JN
.
Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications
.
Blood.
2004
;
104
(
9
):
2623
-
2634
.
17.
Ahmed
R
,
Devasia
AJ
,
Viswabandya
A
, et al
.
Long-term outcome following splenectomy for chronic and persistent immune thrombocytopenia (ITP) in adults and children: splenectomy in ITP
.
Ann Hematol.
2016
;
95
(
9
):
1429
-
1434
.
18.
Boyle
S
,
White
RH
,
Brunson
A
,
Wun
T
.
Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia
.
Blood.
2013
;
121
(
23
):
4782
-
4790
.
19.
Aladjidi
N
,
Leverger
G
,
Leblanc
T
, et al;
Centre de Référence National des Cytopénies Auto-immunes de l’Enfant (CEREVANCE)
.
New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children
.
Haematologica.
2011
;
96
(
5
):
655
-
663
.
20.
Petri
M
,
Orbai
A-M
,
Alarcón
GS
, et al
.
Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus
.
Arthritis Rheum.
2012
;
64
(
8
):
2677
-
2686
.
21.
Bousfiha
A
,
Jeddane
L
,
Picard
C
, et al
.
Human inborn errors of immunity: 2019 update of the IUIS phenotypical classification
.
J Clin Immunol.
2020
;
40
(
1
):
66
-
81
.
22.
Oliveira
JB
,
Bleesing
JJ
,
Dianzani
U
, et al
.
Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop
.
Blood.
2010
;
116
(
14
):
e35
-
e40
.
23.
Tarvin
SE
,
O'Neil
KM
.
Systemic lupus erythematosus, Sjögren syndrome, and mixed connective tissue disease in children and adolescents
.
Pediatr Clin North Am
.
2018
;
65
(
4
):
711
737
.
24.
Kristinsson
SY
,
Gridley
G
,
Hoover
RN
,
Check
D
,
Landgren
O
.
Long-term risks after splenectomy among 8,149 cancer-free American veterans: a cohort study with up to 27 years follow-up
.
Haematologica.
2014
;
99
(
2
):
392
-
398
.
25.
Neven
B
,
Magerus-Chatinet
A
,
Florkin
B
, et al
.
A survey of 90 patients with autoimmune lymphoproliferative syndrome related to TNFRSF6 mutation
.
Blood.
2011
;
118
(
18
):
4798
-
4807
.
26.
Thomsen
RW
,
Schoonen
WM
,
Farkas
DK
,
Riis
A
,
Fryzek
JP
,
Sørensen
HT
.
Risk of venous thromboembolism in splenectomized patients compared with the general population and appendectomized patients: a 10-year nationwide cohort study
.
J Thromb Haemost.
2010
;
8
(
6
):
1413
-
1416
.
27.
Dendle
C
,
Spelman
T
,
Sundararajan
V
,
Chunilal
S
,
Woolley
I
.
An analysis of the thromboembolic outcomes of 2472 splenectomized individuals
.
Blood.
2015
;
125
(
10
):
1681
-
1682
.
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