AUTOIMMUNE HEMOLYTIC ANEMIA IN CHILDHOOD. LONG TERM EXPERIENCE FROM A SINGLE CENTER
(Abstract release date: 05/19/16)
EHA Library. Economou M. 06/09/16; 135043; PB2143
Assoc. Prof. Marina Economou
Contributions
Contributions
Abstract
Abstract: PB2143
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is an immune mediated cytopenia rarely encountered in the first years of life. Childhood AIHA is characterized by diagnostic complexity in many cases and prognostic uncertainty in most, while therapeutic approach remains not well determined.
Aims
Aim of study was to report on the clinical presentation, management and long term outcome of cases with AIHA followed at a single pediatric hematology department during a 12year period.
Methods
The study was retrospective, covering the period between January 2004 and December 2015. Hospital files were used to record data including age at diagnosis, personal and family medical history, laboratory investigation, treatment, time of follow up, complications and relapses.
Results
During the given time period, 16 patients were diagnosed with AIHA. Mean age at diagnosis was 7.8 years (4 months – 13 years) and mean time of follow up 5.08±4.18 years (4 months – 12 years). A family history of autoimmune disorder was reported in 2/16 patients (12.5%). A known history of congenital hemolytic anemia was reported in 3/16 patients (18.7%) and of autoimmune thyroiditis in 1/16 patients (6.25%, while a history of recent infection was recorded in 11/16 patients (68.7%). In 12/16 cases (75%) AIHA was considered primary and in 4/16 (25%) found to be secondary to other conditions - EBV infection in 2 cases, systemic lupus erythematosus in one case and autoimmune lymphoproliferative syndrome in one case. Mean Hb at diagnosis was 6.29 ±2.22 gr/dl and median reticulocyte count 11.2% (0.8 – 32%) - 2 patients initially presenting with reticulocytopenia. In all but 2 cases (93.7%) direct Coombs test was IgG – IgG/C3d positive, in one case IgA positive and in one case initially negative. As to immunology studies, 8 patients presented with transient ANA, 1 patient with findings consistent with SEL (presence of ANA with low C3 and C4) and 2 patients with positive ATA (one already diagnosed with autoimmune thyroiditis). As to management, in 7/16 cases (43.75%) at least one urgent transfusion was required, while in 5/16 cases (31.26%) more than two. All patients received IVIG 1-6g/kg and solu-medrol pulse therapy at least once depending on clinical course, while 4/16 patients (25%) were put on monthly administration for 12 months. Oral prednisolone was administered in 11/16 patients (68.5%) for 3 to 118 months and cyclosporine in 7/16 patients (43.75%) for 3 to 24 months. In total, 6/16 patients (37.5%) required multiple pharmaceutical agents, i.e. IVIG, corticosteroids, cyclosporine and rituximab. As to complications, 4 patients presented with gall bladder lithiasis and 1 with growth retardation and adrenal insufficiency due to chronic corticosteroid use. With regards to clinical course, 3/16 patients (18.7%) presented with an acute course lasting for less than 3 months and 13/16 patients (81.3%) presented with a chronic course. Of these 13 patients, 8 presented with 1 or more (up to 6) relapses during follow-up.
Conclusion
AIHA in children may present with chronic course and unsatisfactory control of hemolysis, requiring prolonged immunosuppressive therapy. In addition, complications related both to disease and treatment may develop.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Childhood
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is an immune mediated cytopenia rarely encountered in the first years of life. Childhood AIHA is characterized by diagnostic complexity in many cases and prognostic uncertainty in most, while therapeutic approach remains not well determined.
Aims
Aim of study was to report on the clinical presentation, management and long term outcome of cases with AIHA followed at a single pediatric hematology department during a 12year period.
Methods
The study was retrospective, covering the period between January 2004 and December 2015. Hospital files were used to record data including age at diagnosis, personal and family medical history, laboratory investigation, treatment, time of follow up, complications and relapses.
Results
During the given time period, 16 patients were diagnosed with AIHA. Mean age at diagnosis was 7.8 years (4 months – 13 years) and mean time of follow up 5.08±4.18 years (4 months – 12 years). A family history of autoimmune disorder was reported in 2/16 patients (12.5%). A known history of congenital hemolytic anemia was reported in 3/16 patients (18.7%) and of autoimmune thyroiditis in 1/16 patients (6.25%, while a history of recent infection was recorded in 11/16 patients (68.7%). In 12/16 cases (75%) AIHA was considered primary and in 4/16 (25%) found to be secondary to other conditions - EBV infection in 2 cases, systemic lupus erythematosus in one case and autoimmune lymphoproliferative syndrome in one case. Mean Hb at diagnosis was 6.29 ±2.22 gr/dl and median reticulocyte count 11.2% (0.8 – 32%) - 2 patients initially presenting with reticulocytopenia. In all but 2 cases (93.7%) direct Coombs test was IgG – IgG/C3d positive, in one case IgA positive and in one case initially negative. As to immunology studies, 8 patients presented with transient ANA, 1 patient with findings consistent with SEL (presence of ANA with low C3 and C4) and 2 patients with positive ATA (one already diagnosed with autoimmune thyroiditis). As to management, in 7/16 cases (43.75%) at least one urgent transfusion was required, while in 5/16 cases (31.26%) more than two. All patients received IVIG 1-6g/kg and solu-medrol pulse therapy at least once depending on clinical course, while 4/16 patients (25%) were put on monthly administration for 12 months. Oral prednisolone was administered in 11/16 patients (68.5%) for 3 to 118 months and cyclosporine in 7/16 patients (43.75%) for 3 to 24 months. In total, 6/16 patients (37.5%) required multiple pharmaceutical agents, i.e. IVIG, corticosteroids, cyclosporine and rituximab. As to complications, 4 patients presented with gall bladder lithiasis and 1 with growth retardation and adrenal insufficiency due to chronic corticosteroid use. With regards to clinical course, 3/16 patients (18.7%) presented with an acute course lasting for less than 3 months and 13/16 patients (81.3%) presented with a chronic course. Of these 13 patients, 8 presented with 1 or more (up to 6) relapses during follow-up.
Conclusion
AIHA in children may present with chronic course and unsatisfactory control of hemolysis, requiring prolonged immunosuppressive therapy. In addition, complications related both to disease and treatment may develop.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Childhood
Abstract: PB2143
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is an immune mediated cytopenia rarely encountered in the first years of life. Childhood AIHA is characterized by diagnostic complexity in many cases and prognostic uncertainty in most, while therapeutic approach remains not well determined.
Aims
Aim of study was to report on the clinical presentation, management and long term outcome of cases with AIHA followed at a single pediatric hematology department during a 12year period.
Methods
The study was retrospective, covering the period between January 2004 and December 2015. Hospital files were used to record data including age at diagnosis, personal and family medical history, laboratory investigation, treatment, time of follow up, complications and relapses.
Results
During the given time period, 16 patients were diagnosed with AIHA. Mean age at diagnosis was 7.8 years (4 months – 13 years) and mean time of follow up 5.08±4.18 years (4 months – 12 years). A family history of autoimmune disorder was reported in 2/16 patients (12.5%). A known history of congenital hemolytic anemia was reported in 3/16 patients (18.7%) and of autoimmune thyroiditis in 1/16 patients (6.25%, while a history of recent infection was recorded in 11/16 patients (68.7%). In 12/16 cases (75%) AIHA was considered primary and in 4/16 (25%) found to be secondary to other conditions - EBV infection in 2 cases, systemic lupus erythematosus in one case and autoimmune lymphoproliferative syndrome in one case. Mean Hb at diagnosis was 6.29 ±2.22 gr/dl and median reticulocyte count 11.2% (0.8 – 32%) - 2 patients initially presenting with reticulocytopenia. In all but 2 cases (93.7%) direct Coombs test was IgG – IgG/C3d positive, in one case IgA positive and in one case initially negative. As to immunology studies, 8 patients presented with transient ANA, 1 patient with findings consistent with SEL (presence of ANA with low C3 and C4) and 2 patients with positive ATA (one already diagnosed with autoimmune thyroiditis). As to management, in 7/16 cases (43.75%) at least one urgent transfusion was required, while in 5/16 cases (31.26%) more than two. All patients received IVIG 1-6g/kg and solu-medrol pulse therapy at least once depending on clinical course, while 4/16 patients (25%) were put on monthly administration for 12 months. Oral prednisolone was administered in 11/16 patients (68.5%) for 3 to 118 months and cyclosporine in 7/16 patients (43.75%) for 3 to 24 months. In total, 6/16 patients (37.5%) required multiple pharmaceutical agents, i.e. IVIG, corticosteroids, cyclosporine and rituximab. As to complications, 4 patients presented with gall bladder lithiasis and 1 with growth retardation and adrenal insufficiency due to chronic corticosteroid use. With regards to clinical course, 3/16 patients (18.7%) presented with an acute course lasting for less than 3 months and 13/16 patients (81.3%) presented with a chronic course. Of these 13 patients, 8 presented with 1 or more (up to 6) relapses during follow-up.
Conclusion
AIHA in children may present with chronic course and unsatisfactory control of hemolysis, requiring prolonged immunosuppressive therapy. In addition, complications related both to disease and treatment may develop.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Childhood
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is an immune mediated cytopenia rarely encountered in the first years of life. Childhood AIHA is characterized by diagnostic complexity in many cases and prognostic uncertainty in most, while therapeutic approach remains not well determined.
Aims
Aim of study was to report on the clinical presentation, management and long term outcome of cases with AIHA followed at a single pediatric hematology department during a 12year period.
Methods
The study was retrospective, covering the period between January 2004 and December 2015. Hospital files were used to record data including age at diagnosis, personal and family medical history, laboratory investigation, treatment, time of follow up, complications and relapses.
Results
During the given time period, 16 patients were diagnosed with AIHA. Mean age at diagnosis was 7.8 years (4 months – 13 years) and mean time of follow up 5.08±4.18 years (4 months – 12 years). A family history of autoimmune disorder was reported in 2/16 patients (12.5%). A known history of congenital hemolytic anemia was reported in 3/16 patients (18.7%) and of autoimmune thyroiditis in 1/16 patients (6.25%, while a history of recent infection was recorded in 11/16 patients (68.7%). In 12/16 cases (75%) AIHA was considered primary and in 4/16 (25%) found to be secondary to other conditions - EBV infection in 2 cases, systemic lupus erythematosus in one case and autoimmune lymphoproliferative syndrome in one case. Mean Hb at diagnosis was 6.29 ±2.22 gr/dl and median reticulocyte count 11.2% (0.8 – 32%) - 2 patients initially presenting with reticulocytopenia. In all but 2 cases (93.7%) direct Coombs test was IgG – IgG/C3d positive, in one case IgA positive and in one case initially negative. As to immunology studies, 8 patients presented with transient ANA, 1 patient with findings consistent with SEL (presence of ANA with low C3 and C4) and 2 patients with positive ATA (one already diagnosed with autoimmune thyroiditis). As to management, in 7/16 cases (43.75%) at least one urgent transfusion was required, while in 5/16 cases (31.26%) more than two. All patients received IVIG 1-6g/kg and solu-medrol pulse therapy at least once depending on clinical course, while 4/16 patients (25%) were put on monthly administration for 12 months. Oral prednisolone was administered in 11/16 patients (68.5%) for 3 to 118 months and cyclosporine in 7/16 patients (43.75%) for 3 to 24 months. In total, 6/16 patients (37.5%) required multiple pharmaceutical agents, i.e. IVIG, corticosteroids, cyclosporine and rituximab. As to complications, 4 patients presented with gall bladder lithiasis and 1 with growth retardation and adrenal insufficiency due to chronic corticosteroid use. With regards to clinical course, 3/16 patients (18.7%) presented with an acute course lasting for less than 3 months and 13/16 patients (81.3%) presented with a chronic course. Of these 13 patients, 8 presented with 1 or more (up to 6) relapses during follow-up.
Conclusion
AIHA in children may present with chronic course and unsatisfactory control of hemolysis, requiring prolonged immunosuppressive therapy. In addition, complications related both to disease and treatment may develop.
Session topic: E-poster
Keyword(s): Autoimmune hemolytic anemia (AIHA), Childhood
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