Book contents
- Frontmatter
- Contents
- List of contributors
- Preface
- Part I Pathophysiology of acquired aplastic anemia
- Part II Epidemiology and clinical features of acquired aplastic anemia
- Part III Treatment of acquired aplastic anemia
- 9 Supportive treatment of patients with severe aplastic anemia
- 10 Immunosuppressive treatment of aplastic anemia
- 11 Role of cytokines in the treatment of aplastic anemia
- 12 HLA-identical sibling bone marrow transplantation to treat severe aplastic anemia
- 13 Alternative donor bone marrow transplantation for severe acquired aplastic anemia
- 14 Treatment of children with acquired aplastic anemia
- 15 Long-term follow-up of patients with aplastic anemia: clonal malignant and nonmalignant complications
- 16 Guidelines for treating aplastic anemia
- Part IV Fanconi's anemia
- Index
12 - HLA-identical sibling bone marrow transplantation to treat severe aplastic anemia
from Part III - Treatment of acquired aplastic anemia
Published online by Cambridge University Press: 18 August 2009
- Frontmatter
- Contents
- List of contributors
- Preface
- Part I Pathophysiology of acquired aplastic anemia
- Part II Epidemiology and clinical features of acquired aplastic anemia
- Part III Treatment of acquired aplastic anemia
- 9 Supportive treatment of patients with severe aplastic anemia
- 10 Immunosuppressive treatment of aplastic anemia
- 11 Role of cytokines in the treatment of aplastic anemia
- 12 HLA-identical sibling bone marrow transplantation to treat severe aplastic anemia
- 13 Alternative donor bone marrow transplantation for severe acquired aplastic anemia
- 14 Treatment of children with acquired aplastic anemia
- 15 Long-term follow-up of patients with aplastic anemia: clonal malignant and nonmalignant complications
- 16 Guidelines for treating aplastic anemia
- Part IV Fanconi's anemia
- Index
Summary
Introduction
Severe aplastic anemia (SAA) is defined by a marrow cellularity of <25% and at least two of the following: (1) a neutrophil count <0.5×109/1; (2) a platelet count <20×109/1; and (3) a reticulocyte count <20×109/1 (SAA Working Party consensus conference). Very severe AA is defined by a neutrophil count of <0.2×109/1.
SAA is rare and in most patients the etiology is unknown (idiopathic SAA). Acquired SAA may be linked to ionizing radiation, chemicals (e.g., benzene), chemotherapeutic agents (International Agranulocytosis and Aplastic Anaemia Study, 1987; Yardley-Jones et al., 1991) or drugs that cause idiosyncratic marrow injury (e.g., gold, chloramphenicol, phenylbutazone and others). Occasionally SAA is associated with viral diseases such as nonA, nonB, nonC hepatitis, parvovirus (pure red cell aplasia) or Epstein–Barr virus and autoimmune disorders such as eosinophilic fasciitis (Fonseca and Tefferi, 1997).
The pathophysiology of SAA is incompletely understood and it is possible that there are various etiologies ultimately presenting as marrow failure. Defective hemopoietic stem cells, a defective marrow microenvironment, impairment of cellular interactions and immunological suppression of marrow function may be involved (Jandl, 1996). A role for autoimmune mechanisms is suggested by the therapeutic response to immunosuppressive treatment (Hathaway et al., 1967; Parkman et al., 1974), and by autologous recovery of marrow function following attempted allogeneic bone marrow transplantation or cyclophosphamide treatment without marrow infusion.
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- Information
- Aplastic AnemiaPathophysiology and Treatment, pp. 230 - 257Publisher: Cambridge University PressPrint publication year: 1999
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