Pure red cell aplasia in autoimmune polyglandular syndrome with T lymphocytosis.
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.
Grossi
A
A
Nozzoli
C
C
Gheri
R
R
Santini
V
V
Marrani
C
C
Zoccolante
A
A
Ferrini
P R
PR
D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy; D001853:Bone Marrow / Q000473:pathology; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008218:Lymphocytosis / Q000150:complications* / Q000188:drug therapy; D008286:Malabsorption Syndromes / Q000150:complications / Q000188:drug therapy; D008297:Male; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications* / Q000188:drug therapy; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D013601:T-Lymphocytes
D007166:Immunosuppressive Agents