| Topic: |
hematologic diseases (he) |
| Title: |
Pathophysiologic Mechanisms in Acquired Aplastic Anemia. |
| Author: |
Young, N.S. |
| Source: |
IN: Hematology 2006. Washington, DC: American Society of Hematology. 2006. pp 72-77. |
| 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 explores the pathophysiologic mechanisms in acquired aplastic anemia, an unusual hematologic disease characterized by an absence of hematopoietic cells. An immune pathophysiology has been inferred for this disease from improvement in blood counts with immunosuppressive therapy in the majority of patients. Molecular mechanisms underlying both T cell effector cells and the target marrow stem and progenitor cells are now being identified. Activated type 1 cytotoxic T cells and type 1 cytokines have been implicated in cell culture experiments; clues to the molecular basis of the aberrant immune response include cytokine gene polymorphisms and abnormalities in the regulatory pathways for gamma-interferon. For stem cell depletion, mutations in genes of the telomere repair complex are present in some patients with apparently acquired aplastic anemia. Telomerase deficiency is associated with short telomeres and a quantitative reduction in marrow progenitors and likely also a qualitative deficiency in the repair capacity of hematopoietic tissue. The author concludes that quantitative and practical measurements of oligoclonal T cell activity and of hematopoietic stem cell number and function may allow laboratory testing to guide treatment decisions. 42 references. |
| Format: |
Book Chapter |
| Language: |
English. |
| Major Keywords: |
Hematologic Diseases. Aplastic Anemia. Pathophysiology. Immune System. |
| Minor Keywords: |
Patient Care Management. Bone Marrow Diseases. Stem Cells. Genetics. Immunosuppressive Agents. |
| Publication Number: |
HEBK10054. |
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