Title:Idiopathic Aplastic Anemia in Children and Adults: Diagnosis, Treatments, and Management - A Review
Volume: 21
Issue: 13
Author(s): Fatmah S. Alqahtany*
Affiliation:
- Department of Pathology, Hematopathology Unit, College of Medicine, King Saud University, King Saud University Medical City, Riyadh,Saudi Arabia
Keywords:
Aplastic anemia, immunosuppression, leukemia, hematologic diseases, pathophysiology, T-cell activation.
Abstract: Aplastic Anemia (AA) is also known as idiopathic aplastic anemia (IAA) and the production
of new blood cells ceases in AA, which leads to an abnormal hematological syndrome such as pancytopenia
and suppression of hypo-cellular bone marrow. The pathophysiology of AA in most cases is
immune-mediated and is stimulated by type 1 cytotoxic T cells. Reliable early diagnostic tests of IAA
are not yet available, therefore most of the cases are identified in advanced stages. Recently, abnormal
immune response and hematopoietic cell deficiencies are defined genetically, such as in target cells of
telomere repair gene mutations and by the dysregulation of T-cell activation pathways. Importantly,
anti-thymocyte globulins and cyclosporine-associated immunosuppression are successful treatments
for restoring blood cell production in most of the cases, however, clonal hematologic diseases remain
challenging. In the current review, we have discussed the common practices in the treatment, pathophysiology,
diagnosis, and management of AA.