Abstract
We report the onset of pure red cell aplasia (PRCA) in a patient with a history of polyglandular syndrome including Addison's disease, malabsorption syndrome, diabetes type I and gastric hyperplastic polyposis. An autoimmune origin for this complex disorder was not supported by the presence of organ specific antibodies, but T cells were found to be of polyclonal origin, as demonstrated by molecular analysis of T cell receptor (TCR) gene rearrangement. The pathophysiology of this case, based on laboratory findings and response to therapy, is discussed.
MeSH terms
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Adult
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Autoimmune Diseases / complications*
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Autoimmune Diseases / drug therapy
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Bone Marrow / pathology
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Gene Rearrangement, beta-Chain T-Cell Antigen Receptor
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Humans
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Immunosuppressive Agents / therapeutic use
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Lymphocytosis / complications*
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Lymphocytosis / drug therapy
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Malabsorption Syndromes / complications
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Malabsorption Syndromes / drug therapy
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Male
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Polyendocrinopathies, Autoimmune / complications*
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Polyendocrinopathies, Autoimmune / drug therapy
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Red-Cell Aplasia, Pure / complications*
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Red-Cell Aplasia, Pure / drug therapy
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Red-Cell Aplasia, Pure / physiopathology
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T-Lymphocytes*