EVANS SYNDROME: A RETROSPECTIVE STUDY OF 30 CASES.
(Abstract release date: 05/19/16)
EHA Library. zahra k. 06/09/16; 134965; PB2065
Dr. kmira zahra
Contributions
Contributions
Abstract
Abstract: PB2065
Type: Publication Only
Background
Evans syndrome is a very rare autoimmune disorder in which an individual's antibodies attack their own red blood cells and platelets. Both of these events may occur simultaneously or one may follow on from the other. Usually idiopathic, it is also associated with lymphoproliferative disorders and autoimmune diseases.
Aims
Study the epidemiological, clinical, biological, etiological and therapeutic aspects of Evans syndrome.
Methods
This is a retrospective and analytic study about 30 cases of Evans syndrome observed in the hematology department of Sousse, over a period of 14 years.
Results
There were 18 men and 12 women (sex ratio = 1,5) with a median age of 40 years. The circumstances of discovery were an anemic syndrome in all patients, mainly due to paleness and asthenia. Concerning biology, regenerative anemia was normocytic in 20 cases and macrocytic in 10 cases, thrombocytopenia below 100000/mm3 was observed in all patients. There were also biological signs of hemolysis: hyperbilirubinemia, high LDH rate in all patients. Direct Coombs test was positive for Ig G + Complement, IgG and complement in 13, 8 and 9 cases, respectively. Evans syndrome was idiopathic in 20 cases and secondary to lymphoproliferative disorders in 5 cases, autoimmune disorders in 5 cases. All patients received corticosteroid treatment in addition to folic acid therapy and etiological treatment in the non idiopathic cases with complete remission in 10 cases. Immunosuppressive therapy (endoxan) was prescribed in 10 patients with complete remission in 5 patients. Anti-CD20 monoclonal antibody was prescribed in 8 patients with complete remission in 4 cases.
Conclusion
Glucocorticoids and/or intravenous immunoglobulins are the mainstay of the treatment in the majority of patients with Evans syndrome. When these treatments fail, patients often require cytotoxic drugs or splenectomy.
Session topic: E-poster
Keyword(s): Anemia, Immunosuppressive therapy, Thrombocythemia
Type: Publication Only
Background
Evans syndrome is a very rare autoimmune disorder in which an individual's antibodies attack their own red blood cells and platelets. Both of these events may occur simultaneously or one may follow on from the other. Usually idiopathic, it is also associated with lymphoproliferative disorders and autoimmune diseases.
Aims
Study the epidemiological, clinical, biological, etiological and therapeutic aspects of Evans syndrome.
Methods
This is a retrospective and analytic study about 30 cases of Evans syndrome observed in the hematology department of Sousse, over a period of 14 years.
Results
There were 18 men and 12 women (sex ratio = 1,5) with a median age of 40 years. The circumstances of discovery were an anemic syndrome in all patients, mainly due to paleness and asthenia. Concerning biology, regenerative anemia was normocytic in 20 cases and macrocytic in 10 cases, thrombocytopenia below 100000/mm3 was observed in all patients. There were also biological signs of hemolysis: hyperbilirubinemia, high LDH rate in all patients. Direct Coombs test was positive for Ig G + Complement, IgG and complement in 13, 8 and 9 cases, respectively. Evans syndrome was idiopathic in 20 cases and secondary to lymphoproliferative disorders in 5 cases, autoimmune disorders in 5 cases. All patients received corticosteroid treatment in addition to folic acid therapy and etiological treatment in the non idiopathic cases with complete remission in 10 cases. Immunosuppressive therapy (endoxan) was prescribed in 10 patients with complete remission in 5 patients. Anti-CD20 monoclonal antibody was prescribed in 8 patients with complete remission in 4 cases.
Conclusion
Glucocorticoids and/or intravenous immunoglobulins are the mainstay of the treatment in the majority of patients with Evans syndrome. When these treatments fail, patients often require cytotoxic drugs or splenectomy.
Session topic: E-poster
Keyword(s): Anemia, Immunosuppressive therapy, Thrombocythemia
Abstract: PB2065
Type: Publication Only
Background
Evans syndrome is a very rare autoimmune disorder in which an individual's antibodies attack their own red blood cells and platelets. Both of these events may occur simultaneously or one may follow on from the other. Usually idiopathic, it is also associated with lymphoproliferative disorders and autoimmune diseases.
Aims
Study the epidemiological, clinical, biological, etiological and therapeutic aspects of Evans syndrome.
Methods
This is a retrospective and analytic study about 30 cases of Evans syndrome observed in the hematology department of Sousse, over a period of 14 years.
Results
There were 18 men and 12 women (sex ratio = 1,5) with a median age of 40 years. The circumstances of discovery were an anemic syndrome in all patients, mainly due to paleness and asthenia. Concerning biology, regenerative anemia was normocytic in 20 cases and macrocytic in 10 cases, thrombocytopenia below 100000/mm3 was observed in all patients. There were also biological signs of hemolysis: hyperbilirubinemia, high LDH rate in all patients. Direct Coombs test was positive for Ig G + Complement, IgG and complement in 13, 8 and 9 cases, respectively. Evans syndrome was idiopathic in 20 cases and secondary to lymphoproliferative disorders in 5 cases, autoimmune disorders in 5 cases. All patients received corticosteroid treatment in addition to folic acid therapy and etiological treatment in the non idiopathic cases with complete remission in 10 cases. Immunosuppressive therapy (endoxan) was prescribed in 10 patients with complete remission in 5 patients. Anti-CD20 monoclonal antibody was prescribed in 8 patients with complete remission in 4 cases.
Conclusion
Glucocorticoids and/or intravenous immunoglobulins are the mainstay of the treatment in the majority of patients with Evans syndrome. When these treatments fail, patients often require cytotoxic drugs or splenectomy.
Session topic: E-poster
Keyword(s): Anemia, Immunosuppressive therapy, Thrombocythemia
Type: Publication Only
Background
Evans syndrome is a very rare autoimmune disorder in which an individual's antibodies attack their own red blood cells and platelets. Both of these events may occur simultaneously or one may follow on from the other. Usually idiopathic, it is also associated with lymphoproliferative disorders and autoimmune diseases.
Aims
Study the epidemiological, clinical, biological, etiological and therapeutic aspects of Evans syndrome.
Methods
This is a retrospective and analytic study about 30 cases of Evans syndrome observed in the hematology department of Sousse, over a period of 14 years.
Results
There were 18 men and 12 women (sex ratio = 1,5) with a median age of 40 years. The circumstances of discovery were an anemic syndrome in all patients, mainly due to paleness and asthenia. Concerning biology, regenerative anemia was normocytic in 20 cases and macrocytic in 10 cases, thrombocytopenia below 100000/mm3 was observed in all patients. There were also biological signs of hemolysis: hyperbilirubinemia, high LDH rate in all patients. Direct Coombs test was positive for Ig G + Complement, IgG and complement in 13, 8 and 9 cases, respectively. Evans syndrome was idiopathic in 20 cases and secondary to lymphoproliferative disorders in 5 cases, autoimmune disorders in 5 cases. All patients received corticosteroid treatment in addition to folic acid therapy and etiological treatment in the non idiopathic cases with complete remission in 10 cases. Immunosuppressive therapy (endoxan) was prescribed in 10 patients with complete remission in 5 patients. Anti-CD20 monoclonal antibody was prescribed in 8 patients with complete remission in 4 cases.
Conclusion
Glucocorticoids and/or intravenous immunoglobulins are the mainstay of the treatment in the majority of patients with Evans syndrome. When these treatments fail, patients often require cytotoxic drugs or splenectomy.
Session topic: E-poster
Keyword(s): Anemia, Immunosuppressive therapy, Thrombocythemia
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