TY - JOUR
T1 - Practical aspects in the diagnosis and management of aplastic anemia
AU - Fonseca, Rafael
AU - Tefferi, Ayalew
PY - 1997
Y1 - 1997
N2 - Aplastic anemia may result from several pathogenic mechanisms, the most common is idiopathic. The current definitive treatments for aplastic anemia are bone marrow transplantation (BMT) or immunosuppressive (IS) therapy. The benefits of each are comparable. However, certain subsets of patients derive superior benefit from one of the other. Bone marrow transplantation is the initial treatment of choice for young patients (<20 years old). It results in the complete reconstitution of hematopoiesis, whereas autologous hematopoietic remissions after IS therapy are more susceptible to relapse. Survival rates after BMT, in patients between the ages of 20 and 40, are comparable to those reported for IS therapy. Better survival rates after BMT have been achieved with improved conditioning regimens and graft-versus-host disease prophylaxis. For patients older than 40, the treatment of choice is IS. Long-term complications of IS therapy include recurrence and development of clonal myeloid disorders. Long-term complications after BMT include graft- versus host disease and secondary neoplasms. The IS regimen includes the combination of antithymocyte globulin and cyclosporin A. The addition of growth factor to the IS regimen seems promising; however, their use on their own is not recommended. Androgens have been shown to be inferior in the treatment of aplastic anemia. The role of BMT from an unrelated donor is being investigated.
AB - Aplastic anemia may result from several pathogenic mechanisms, the most common is idiopathic. The current definitive treatments for aplastic anemia are bone marrow transplantation (BMT) or immunosuppressive (IS) therapy. The benefits of each are comparable. However, certain subsets of patients derive superior benefit from one of the other. Bone marrow transplantation is the initial treatment of choice for young patients (<20 years old). It results in the complete reconstitution of hematopoiesis, whereas autologous hematopoietic remissions after IS therapy are more susceptible to relapse. Survival rates after BMT, in patients between the ages of 20 and 40, are comparable to those reported for IS therapy. Better survival rates after BMT have been achieved with improved conditioning regimens and graft-versus-host disease prophylaxis. For patients older than 40, the treatment of choice is IS. Long-term complications of IS therapy include recurrence and development of clonal myeloid disorders. Long-term complications after BMT include graft- versus host disease and secondary neoplasms. The IS regimen includes the combination of antithymocyte globulin and cyclosporin A. The addition of growth factor to the IS regimen seems promising; however, their use on their own is not recommended. Androgens have been shown to be inferior in the treatment of aplastic anemia. The role of BMT from an unrelated donor is being investigated.
KW - Bone marrow transplantation
KW - Diagnosis
KW - Immunosuppressive therapy
KW - Outcome
KW - Survival rate
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U2 - 10.1097/00000441-199703000-00007
DO - 10.1097/00000441-199703000-00007
M3 - Review article
C2 - 9075433
AN - SCOPUS:0030893461
SN - 0002-9629
VL - 313
SP - 159
EP - 169
JO - American Journal of the Medical Sciences
JF - American Journal of the Medical Sciences
IS - 3
ER -