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==Overview==
==Overview==
Patients with this disorder have pancytopenia, a low reticulocyte count, and a hypoplastic bone marrow (<20% cellularity) with normal maturation of all cell lines. Aplastic anemia is a fatal disorder in which myeloid progenitor cells and stem cells are severely diminished or absent in the bone marrow because of either an intrinsic defect of the stem cells or immune-mediated stem cell destruction, which leads to transfusion-dependent anemia, thrombocytopenia, and severe neutropenia. In approximately 50% of cases of aplastic anemia, there
is no obvious cause; chemicals, drugs, viral infections, collagen vascular diseases, and thymoma can be implicated in the remaining cases. Interferon-activated T lymphocytes are involved in autoimmune destruction of stem cells in a significant proportion of patients with the idiopathic or the acquired form of the disease; this fact explains why immunosuppressive therapy is effective in some patients. Initial management involves withdrawal of any potentially causative agents and a CT scan of the chest to rule out an associated thymoma.
==Laboratory Findings==
==Laboratory Findings==
===Electrolyte and Biomarker Studies===
===Electrolyte and Biomarker Studies===

Revision as of 12:33, 23 September 2012

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Editor-In-Chief: Aric Hall, M.D., Beth Israel Deaconess Medical Center, Boston, MA [1]

Overview

Patients with this disorder have pancytopenia, a low reticulocyte count, and a hypoplastic bone marrow (<20% cellularity) with normal maturation of all cell lines. Aplastic anemia is a fatal disorder in which myeloid progenitor cells and stem cells are severely diminished or absent in the bone marrow because of either an intrinsic defect of the stem cells or immune-mediated stem cell destruction, which leads to transfusion-dependent anemia, thrombocytopenia, and severe neutropenia. In approximately 50% of cases of aplastic anemia, there is no obvious cause; chemicals, drugs, viral infections, collagen vascular diseases, and thymoma can be implicated in the remaining cases. Interferon-activated T lymphocytes are involved in autoimmune destruction of stem cells in a significant proportion of patients with the idiopathic or the acquired form of the disease; this fact explains why immunosuppressive therapy is effective in some patients. Initial management involves withdrawal of any potentially causative agents and a CT scan of the chest to rule out an associated thymoma.

Laboratory Findings

Electrolyte and Biomarker Studies

References