Abstract
An 81-year-old female was diagnosed with Hashimoto's thyroiditis in 1998. In 2002, she developed diabetes mellitus with blood glucose and HbA1c levels of 508 mg/dl and 12.1 % (JDS), respectively. Initially, she was treated with insulin and subsequently with oral hypoglycemic agents, achieving improved blood glucose control. In 2005, pancytopenia was noticed, and a diagnosis of myelodysplastic syndrome was made. In 2008, due to poor glycemic control, the treatment was switched to insulin therapy. In 2009, she was admitted to our hospital because of progressive anemia and poor glycemic control, and the further analyses confirmed a diagnosis of type 1 diabetes mellitus with low endogenous insulin and a positive anti-GAD antibody titer. After being discharged, the patient showed macrocytic anemia with low serum VitB12 and autoantibodies against intrinsic factor and gastric parietal cells, resulting in a diagnosis of pernicious anemia. The co-existence of type 1 diabetes, Hashimoto's thyroiditis and pernicious anemia indicates the presence of polyglandular autoimmune syndrome type III-A. We herein report a rare case of polyglandular autoimmune syndrome type III-A associated with a strong family history of autoimmune thyroid diseases complicated with myelodysplastic syndrome, which has accumulating evidence of an association with autoimmunity. The analyses of HLA and CTLA4 genotypes, as well as a literature review, are described.
