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| Topic: |
hematologic diseases (he) |
| Title: |
Making Therapeutic Decisions in Adults with Aplastic Anemia. |
| Author: |
Marsh, J. |
| Source: |
IN: Hematology 2006. Washington, DC: American Society of Hematology. 2006. pp 78-85. |
| Availability: |
Available from American Society of Hematology. 1900 M Street NW, Suite 200, Washington, DC 20036. Email: ASH@hematology.org. Website: www.asheducationbook.org. Price: Available free on website. |
| Abstract: |
This article reviews the therapeutic management of adults with aplastic anemia (AA). The author recommends careful exclusion of other causes of bone marrow failure and cautions that late-onset inherited forms of AA may present in adulthood with subclinical disease. Recent long-term studies of HLA-identical sibling donor bone marrow transplantation (BMT) show excellent survival for patients under the age of 40 years, but chronic graft-versus-host disease (GVHD) is still a major problem, particularly for quality of life. Recent improvements in outcome after matched, unrelated-donor BMT may reflect better donor matching and use of reduced intensity conditioning regimens. For patients treated with immunosuppressive therapy, antithymocyte globulin (ATG) and cyclosporin (CSA) remain the standard regimen with excellent overall survival but less impressive failure-free survival due to nonresponse, relapse, and later clonal disorders. The benefit of adding granulocyte colony-stimulating factor (G-CSF) to ATG and CSA is unclear and being assessed in a further prospective European study. The author notes that patients who are refractory to conventional immunosuppressive therapy and currently ineligible for BMT represent difficult management problems. For this patient population, new approaches to transplantation are being evaluated, such as fludarabine-based conditioning regimens and the potential use of double umbilical cord blood transplants, but there is a need for new immunosuppressive agents. The author concludes that improved supportive care is likely to be a major factor in better outcome for all AA patients regardless of treatment approach. 1 figure. 2 tables. 40 references. |
| Format: |
Book Chapter |
| Language: |
English. |
| Major Keywords: |
Hematologic Diseases. Aplastic Anemia. Adults. Patient Care Management. Bone Marrow. Transplantation. Immunosuppressive Agents. |
| Minor Keywords: |
Immune System. Bone Marrow Diseases. Histocompatibility. Graft Versus Host Disease. Drug Therapy. Drug Effects. |
| Publication Number: |
HEBK10055. |
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