RESULTS OF STANDARD TREATMENT FOR APLASTIC ANEMIA IN CHILDREN
(Abstract release date: 05/19/16)
EHA Library. Vaskina E. 06/09/16; 134960; PB2060
Mrs. Elena Vaskina
Contributions
Contributions
Abstract
Abstract: PB2060
Type: Publication Only
Background
Acquired aplastic anemia (AA) is a disorder characterized by pancytopenia, hypocellular bone marrow and an absence of infiltrative disease of the bone marrow. The incidence of AA in Russian Federation is 2-6 cases among 1 million children per year. The hematopoietic stem cell transplantation (HSCT) is the most effective treatment for AA. Immunosuppressive therapy (IST) including antilymphocyte globulin, cyclosporine is the standard therapy for patients who do not have a HLA-matched sibling donor.
Aims
Evaluation of results of treatment of children with acquired aplastic anemia in 2005-2015.
Methods
The study included 23 children with acquired aplastic anemia at the age from 3 to 18 years (median age - 11 years): 11 boys and 12 girls. Very severe disease was diagnosed in 12 patients, severe AA - in 8, non-severe AA – in three patients. The hemorrhagic syndrome with uterine, nasal and intestinal bleeding was observed in 25% of patients.
Results
Remission was achieved after the first course of IST in 12 patients. The second course of IST was held in 5 patients with very severe AA, including as a therapy for relapse in three patients. Early hematologic relapse was diagnosed in 2 patients, the later relapse - in 1 patient. HSCT from an related donor carried two children after the first course of ICT. Unrelated donor HSCT was performed in two patients after the second course of IST. Five patients at this time continue to receive cyclosporin therapy, two of these patients are dependent on transfusional support. One child was killed in a month from the beginning of ICT. Cause of death - sepsis, severe hemorrhagic syndrome. On 01.01.2016, 17 patients (77%) are in complete clinical remission.
Conclusion
Five year overall survival rate 95,6%. Event-free five year survival rate 80,3%.
Session topic: E-poster
Keyword(s): Aplastic anemia, HSCT, Immunosuppressive therapy, Survival
Type: Publication Only
Background
Acquired aplastic anemia (AA) is a disorder characterized by pancytopenia, hypocellular bone marrow and an absence of infiltrative disease of the bone marrow. The incidence of AA in Russian Federation is 2-6 cases among 1 million children per year. The hematopoietic stem cell transplantation (HSCT) is the most effective treatment for AA. Immunosuppressive therapy (IST) including antilymphocyte globulin, cyclosporine is the standard therapy for patients who do not have a HLA-matched sibling donor.
Aims
Evaluation of results of treatment of children with acquired aplastic anemia in 2005-2015.
Methods
The study included 23 children with acquired aplastic anemia at the age from 3 to 18 years (median age - 11 years): 11 boys and 12 girls. Very severe disease was diagnosed in 12 patients, severe AA - in 8, non-severe AA – in three patients. The hemorrhagic syndrome with uterine, nasal and intestinal bleeding was observed in 25% of patients.
Results
Remission was achieved after the first course of IST in 12 patients. The second course of IST was held in 5 patients with very severe AA, including as a therapy for relapse in three patients. Early hematologic relapse was diagnosed in 2 patients, the later relapse - in 1 patient. HSCT from an related donor carried two children after the first course of ICT. Unrelated donor HSCT was performed in two patients after the second course of IST. Five patients at this time continue to receive cyclosporin therapy, two of these patients are dependent on transfusional support. One child was killed in a month from the beginning of ICT. Cause of death - sepsis, severe hemorrhagic syndrome. On 01.01.2016, 17 patients (77%) are in complete clinical remission.
Conclusion
Five year overall survival rate 95,6%. Event-free five year survival rate 80,3%.
Session topic: E-poster
Keyword(s): Aplastic anemia, HSCT, Immunosuppressive therapy, Survival
Abstract: PB2060
Type: Publication Only
Background
Acquired aplastic anemia (AA) is a disorder characterized by pancytopenia, hypocellular bone marrow and an absence of infiltrative disease of the bone marrow. The incidence of AA in Russian Federation is 2-6 cases among 1 million children per year. The hematopoietic stem cell transplantation (HSCT) is the most effective treatment for AA. Immunosuppressive therapy (IST) including antilymphocyte globulin, cyclosporine is the standard therapy for patients who do not have a HLA-matched sibling donor.
Aims
Evaluation of results of treatment of children with acquired aplastic anemia in 2005-2015.
Methods
The study included 23 children with acquired aplastic anemia at the age from 3 to 18 years (median age - 11 years): 11 boys and 12 girls. Very severe disease was diagnosed in 12 patients, severe AA - in 8, non-severe AA – in three patients. The hemorrhagic syndrome with uterine, nasal and intestinal bleeding was observed in 25% of patients.
Results
Remission was achieved after the first course of IST in 12 patients. The second course of IST was held in 5 patients with very severe AA, including as a therapy for relapse in three patients. Early hematologic relapse was diagnosed in 2 patients, the later relapse - in 1 patient. HSCT from an related donor carried two children after the first course of ICT. Unrelated donor HSCT was performed in two patients after the second course of IST. Five patients at this time continue to receive cyclosporin therapy, two of these patients are dependent on transfusional support. One child was killed in a month from the beginning of ICT. Cause of death - sepsis, severe hemorrhagic syndrome. On 01.01.2016, 17 patients (77%) are in complete clinical remission.
Conclusion
Five year overall survival rate 95,6%. Event-free five year survival rate 80,3%.
Session topic: E-poster
Keyword(s): Aplastic anemia, HSCT, Immunosuppressive therapy, Survival
Type: Publication Only
Background
Acquired aplastic anemia (AA) is a disorder characterized by pancytopenia, hypocellular bone marrow and an absence of infiltrative disease of the bone marrow. The incidence of AA in Russian Federation is 2-6 cases among 1 million children per year. The hematopoietic stem cell transplantation (HSCT) is the most effective treatment for AA. Immunosuppressive therapy (IST) including antilymphocyte globulin, cyclosporine is the standard therapy for patients who do not have a HLA-matched sibling donor.
Aims
Evaluation of results of treatment of children with acquired aplastic anemia in 2005-2015.
Methods
The study included 23 children with acquired aplastic anemia at the age from 3 to 18 years (median age - 11 years): 11 boys and 12 girls. Very severe disease was diagnosed in 12 patients, severe AA - in 8, non-severe AA – in three patients. The hemorrhagic syndrome with uterine, nasal and intestinal bleeding was observed in 25% of patients.
Results
Remission was achieved after the first course of IST in 12 patients. The second course of IST was held in 5 patients with very severe AA, including as a therapy for relapse in three patients. Early hematologic relapse was diagnosed in 2 patients, the later relapse - in 1 patient. HSCT from an related donor carried two children after the first course of ICT. Unrelated donor HSCT was performed in two patients after the second course of IST. Five patients at this time continue to receive cyclosporin therapy, two of these patients are dependent on transfusional support. One child was killed in a month from the beginning of ICT. Cause of death - sepsis, severe hemorrhagic syndrome. On 01.01.2016, 17 patients (77%) are in complete clinical remission.
Conclusion
Five year overall survival rate 95,6%. Event-free five year survival rate 80,3%.
Session topic: E-poster
Keyword(s): Aplastic anemia, HSCT, Immunosuppressive therapy, Survival
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