A Case of Polyglandular Autoimmune Syndrome Type III-A and Myelodysplastic Syndrome with a Strong Family History of Autoimmune Thyroid Diseases

DOI Web Site 23 References

Bibliographic Information

Other Title
  • 家系内に自己免疫性甲状腺疾患が多発し，多腺性自己免疫症候群III-A型に骨髄異形成症候群を合併した1例
  • ショウレイ ホウコク カケイ ナイ ニ ジコ メンエキセイ コウジョウセン シッカン ガ タハツ シ,タセンセイ ジコ メンエキ ショウコウグン Ⅲ-Aガタ ニ コツズイイケイセイ ショウコウグン オ ガッペイ シタ 1レイ

Search this article

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.<br>

Journal

References(23)*help

See more

Related Projects

See more

Keywords

Details 詳細情報について

Report a problem

Back to top