PROSPECTIVE STUDY OF SPLENECTOMY IN AUTO-IMMUNE CYTOPENIA OF CHILDREN
(Abstract release date: 05/19/16)
EHA Library. Aladjidi N. 06/09/16; 132956; E1407
Dr. Nathalie Aladjidi
Contributions
Contributions
Abstract
Abstract: E1407
Type: Eposter Presentation
Background
Chronic immune thrombocytopenic purpura (cITP), autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES) are rare autoimmune diseases in children, characterized in 20-70% of cases by recurrent outbreaks and treatment dependence. In AIHA and ES, the effectiveness of splenectomy has never been clearly established, as it was in ITP. Since 2004, the OBS’CEREVANCE cohort has been recording all the cases of auto-immune cytopenias in children treated in the 30 French pediatric hematology units.
Aims
This prospective observational national study reports the practice, efficacy and safety of splenectomy performed for all the patients of this cohort.
Methods
On 1st January 2016, patients recorded in this cohort who underwent splenectomy in second-line after immunoglobulin and/or steroid failure, before the age of 18, were analyzed in 3 homogeneous groups according to the target: isolated cITP, isolated AIHA, and ES.
Results
Among the 1094 children included in the cohort, diagnosed between 1986 and 2014 (556 isolated cITP, 356 isolated AIHA and 172 ES), 181 underwent a splenectomy.86 of those splenectomies were performed in third or more line in heterogeneous patients. 95 splenectomies were carried out on second-line, 80 for isolated cITP, 8 for isolated AIHA and 7 for ES. Laparoscopy was realized in 79% of cases. The median age at splenectomy was respectively 11.5, 5.9 and 8.4 years. The median delay from diagnosis to splenectomy was respectively 2.3, 0.7 and 1.8 years. The complete response achievement rate was similar in the 3 groups. With a median follow-up of 5 years post splenectomy, there was no need for further immunosuppressive treatment for 64/80 cITP (80%), 4/8 AIHA (50%) and 1/7 SE (15%). Of these 69 patients, 84% were still in continuous complete remission at the last follow-up (54 cITP, 4 AIHA, 0 ES). Comparative relapse-free survival curves are presented. Three of the four deaths are possibly related to the weight of the treatments. Infections and thrombosis were more frequent in AIHA and ES than in cITP.
Conclusion
This prospective national study provides the first evidence-based comparative data on children with early splenectomy for those rare diseases. The benefit-risk ratio of splenectomy seems lower in AIHA and SE than in cITP. Some underlying immune deficiencies as autoimmune lymphoproliferative syndrome are formal contraindications. Prospective pediatric studies will refine alternative immunomodulatory strategies aiming to delay splenectomy as late as possible in children with AIHA or ES.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Idiopathic thombocytopenic purpura (ITP), Pediatric, Splenectomy
Type: Eposter Presentation
Background
Chronic immune thrombocytopenic purpura (cITP), autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES) are rare autoimmune diseases in children, characterized in 20-70% of cases by recurrent outbreaks and treatment dependence. In AIHA and ES, the effectiveness of splenectomy has never been clearly established, as it was in ITP. Since 2004, the OBS’CEREVANCE cohort has been recording all the cases of auto-immune cytopenias in children treated in the 30 French pediatric hematology units.
Aims
This prospective observational national study reports the practice, efficacy and safety of splenectomy performed for all the patients of this cohort.
Methods
On 1st January 2016, patients recorded in this cohort who underwent splenectomy in second-line after immunoglobulin and/or steroid failure, before the age of 18, were analyzed in 3 homogeneous groups according to the target: isolated cITP, isolated AIHA, and ES.
Results
Among the 1094 children included in the cohort, diagnosed between 1986 and 2014 (556 isolated cITP, 356 isolated AIHA and 172 ES), 181 underwent a splenectomy.86 of those splenectomies were performed in third or more line in heterogeneous patients. 95 splenectomies were carried out on second-line, 80 for isolated cITP, 8 for isolated AIHA and 7 for ES. Laparoscopy was realized in 79% of cases. The median age at splenectomy was respectively 11.5, 5.9 and 8.4 years. The median delay from diagnosis to splenectomy was respectively 2.3, 0.7 and 1.8 years. The complete response achievement rate was similar in the 3 groups. With a median follow-up of 5 years post splenectomy, there was no need for further immunosuppressive treatment for 64/80 cITP (80%), 4/8 AIHA (50%) and 1/7 SE (15%). Of these 69 patients, 84% were still in continuous complete remission at the last follow-up (54 cITP, 4 AIHA, 0 ES). Comparative relapse-free survival curves are presented. Three of the four deaths are possibly related to the weight of the treatments. Infections and thrombosis were more frequent in AIHA and ES than in cITP.
Conclusion
This prospective national study provides the first evidence-based comparative data on children with early splenectomy for those rare diseases. The benefit-risk ratio of splenectomy seems lower in AIHA and SE than in cITP. Some underlying immune deficiencies as autoimmune lymphoproliferative syndrome are formal contraindications. Prospective pediatric studies will refine alternative immunomodulatory strategies aiming to delay splenectomy as late as possible in children with AIHA or ES.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Idiopathic thombocytopenic purpura (ITP), Pediatric, Splenectomy
Abstract: E1407
Type: Eposter Presentation
Background
Chronic immune thrombocytopenic purpura (cITP), autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES) are rare autoimmune diseases in children, characterized in 20-70% of cases by recurrent outbreaks and treatment dependence. In AIHA and ES, the effectiveness of splenectomy has never been clearly established, as it was in ITP. Since 2004, the OBS’CEREVANCE cohort has been recording all the cases of auto-immune cytopenias in children treated in the 30 French pediatric hematology units.
Aims
This prospective observational national study reports the practice, efficacy and safety of splenectomy performed for all the patients of this cohort.
Methods
On 1st January 2016, patients recorded in this cohort who underwent splenectomy in second-line after immunoglobulin and/or steroid failure, before the age of 18, were analyzed in 3 homogeneous groups according to the target: isolated cITP, isolated AIHA, and ES.
Results
Among the 1094 children included in the cohort, diagnosed between 1986 and 2014 (556 isolated cITP, 356 isolated AIHA and 172 ES), 181 underwent a splenectomy.86 of those splenectomies were performed in third or more line in heterogeneous patients. 95 splenectomies were carried out on second-line, 80 for isolated cITP, 8 for isolated AIHA and 7 for ES. Laparoscopy was realized in 79% of cases. The median age at splenectomy was respectively 11.5, 5.9 and 8.4 years. The median delay from diagnosis to splenectomy was respectively 2.3, 0.7 and 1.8 years. The complete response achievement rate was similar in the 3 groups. With a median follow-up of 5 years post splenectomy, there was no need for further immunosuppressive treatment for 64/80 cITP (80%), 4/8 AIHA (50%) and 1/7 SE (15%). Of these 69 patients, 84% were still in continuous complete remission at the last follow-up (54 cITP, 4 AIHA, 0 ES). Comparative relapse-free survival curves are presented. Three of the four deaths are possibly related to the weight of the treatments. Infections and thrombosis were more frequent in AIHA and ES than in cITP.
Conclusion
This prospective national study provides the first evidence-based comparative data on children with early splenectomy for those rare diseases. The benefit-risk ratio of splenectomy seems lower in AIHA and SE than in cITP. Some underlying immune deficiencies as autoimmune lymphoproliferative syndrome are formal contraindications. Prospective pediatric studies will refine alternative immunomodulatory strategies aiming to delay splenectomy as late as possible in children with AIHA or ES.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Idiopathic thombocytopenic purpura (ITP), Pediatric, Splenectomy
Type: Eposter Presentation
Background
Chronic immune thrombocytopenic purpura (cITP), autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES) are rare autoimmune diseases in children, characterized in 20-70% of cases by recurrent outbreaks and treatment dependence. In AIHA and ES, the effectiveness of splenectomy has never been clearly established, as it was in ITP. Since 2004, the OBS’CEREVANCE cohort has been recording all the cases of auto-immune cytopenias in children treated in the 30 French pediatric hematology units.
Aims
This prospective observational national study reports the practice, efficacy and safety of splenectomy performed for all the patients of this cohort.
Methods
On 1st January 2016, patients recorded in this cohort who underwent splenectomy in second-line after immunoglobulin and/or steroid failure, before the age of 18, were analyzed in 3 homogeneous groups according to the target: isolated cITP, isolated AIHA, and ES.
Results
Among the 1094 children included in the cohort, diagnosed between 1986 and 2014 (556 isolated cITP, 356 isolated AIHA and 172 ES), 181 underwent a splenectomy.86 of those splenectomies were performed in third or more line in heterogeneous patients. 95 splenectomies were carried out on second-line, 80 for isolated cITP, 8 for isolated AIHA and 7 for ES. Laparoscopy was realized in 79% of cases. The median age at splenectomy was respectively 11.5, 5.9 and 8.4 years. The median delay from diagnosis to splenectomy was respectively 2.3, 0.7 and 1.8 years. The complete response achievement rate was similar in the 3 groups. With a median follow-up of 5 years post splenectomy, there was no need for further immunosuppressive treatment for 64/80 cITP (80%), 4/8 AIHA (50%) and 1/7 SE (15%). Of these 69 patients, 84% were still in continuous complete remission at the last follow-up (54 cITP, 4 AIHA, 0 ES). Comparative relapse-free survival curves are presented. Three of the four deaths are possibly related to the weight of the treatments. Infections and thrombosis were more frequent in AIHA and ES than in cITP.
Conclusion
This prospective national study provides the first evidence-based comparative data on children with early splenectomy for those rare diseases. The benefit-risk ratio of splenectomy seems lower in AIHA and SE than in cITP. Some underlying immune deficiencies as autoimmune lymphoproliferative syndrome are formal contraindications. Prospective pediatric studies will refine alternative immunomodulatory strategies aiming to delay splenectomy as late as possible in children with AIHA or ES.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Idiopathic thombocytopenic purpura (ITP), Pediatric, Splenectomy
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