Yonsei Med J.  2006 Jun;47(3):432-436. 10.3349/ymj.2006.47.3.432.

A 20-Year-Old Woman with Hashimoto's Thyroiditis and Evans' Syndrome

• Kang MY
• Hahm JR
• Jung TS
• Lee GW
• Kim DR
• Park MH

• KMID: 1715865
• DOI: http://doi.org/10.3349/ymj.2006.47.3.432

Abstract

Here we report the case of a 20-year-old female patient previously diagnosed with Hashimoto's thyroiditis and overt hypothyroidism, and who had been taking synthetic thyroxine (100micro/day) for eight months. She experienced intermittent dizziness and generalized weakness, and was diagnosed as having severe autoimmune hemolytic anemia (AIHA). We prescribed prednisolone treatment and continued synthetic thyroxine administration. Two years and five months later, she developed idiopathic thrombocytopenic purpura (ITP) and was diagnosed with Evans' syndrome. Thereafter, laparoscopic splenectomy was performed because her autoimmune hemolytic anemia was refractory and dependent on steroid therapy. The HLA genotypes of the patient were HLA-A*020101/A*2602, HLA-B*270502/B*5401, HLA-Cw*0102/Cw*020202, HLA-DRB1*0404/DRB1*0405, and HLA-DQB1*0302/DQ B1*0401. Hashimoto's thyroiditis is often associated with other nonendocrine autoimmune diseases, and antithyroid antibodies are frequently observed in Evans' syndrome (coexistence of AIHA and ITP). However, there is no report of Evans' syndrome developing in patients with overt hypothyroidism and Hashimoto's thyroiditis. This case suggests that three autoimmune diseases (AIHA, ITP, and Hashimoto's thyroiditis) might share a common immunogenetic pathway in pathogenesis.

Keyword

Thyroiditis; anemia; hemolytic; autoimmunity; purpura; thrombocytopenia

MeSH Terms

Purpura, Thrombocytopenic/blood/*complications
Humans
Hashimoto Disease/*complications/radionuclide imaging
Female
Anemia, Hemolytic, Autoimmune/blood/*complications
Adult

Figure

• 

  Fig. 1 Peripheral blood smear on admission. The peripheral blood smear shows typical hemolytic anemia features such as spherocytosis, poikilocytosis, macrocytosis, and polychromasia of red blood cells, and shows normal findings for white blood cells and platelets (Wright stain, ×400).

• 

  Fig. 2 The technetium-99m thyroid scan. The thyroid gland is slightly enlarged with an inhomogenous uptake pattern. Uptake ratio of the thyroid gland is 2.8%.

Reference

1. Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med. 1951. 87:48–65.

2. Branehög I, Olsson KS, Weinfeld A, Domellöf L. Association of hyperthyroidism with idiopathic thrombocytopenic purpura and haemolytic anaemia. Acta Med Scand. 1979. 205:125–131.

3. Hiraoka N, Setoguchi J, Gotoh I. A case of complication of Evans' syndrome and Basedow's disease. Matsujinkai M J. 1988. 27:79. (in Japanese).

4. Ito M, Ninomiya N, Mizuno H, Ohota H. A case of Evans' syndrome combination of hyperthyroidism. J Jpn Soc Intern Med. 1985. 74:1615. (in Japanese).

5. Sawada Y, Yaombomatani T, Kuroe K, Kanazawa T, Kawazu S. A case of suspicion of Evans' syndrome aggravating anemia and thrombocytopenia associated with hyperthyroid function. Jpn J Clin Haematol. 1989. 30:1642. (in Japanese).

6. Sakai Y, Honda K, Tominaga J, Mitsuwa T, Miyazawa K, Uchida H, et al. A case of Evans' syndrome combination of Graves' disease. Jpn J Clin Endocrinol. 1991. 39:64–66. (in Japanese).

7. Lee FY, Ho CH, Chong LL. Hyperthyroidism and Evans' syndrome. A case report. J Formosan Med Assoc. 1985. 84:256–260.

8. Yashiro M, Nagoshi H, Kasuga Y, Isobe H, Kitajima S, Nakagawa T, et al. Evans' syndrome associated with Graves' disease. Intern Med. 1996. 35:987–990.

9. Ikeda K, Maruyama Y, Yokoyama M, Kato N, Yamanoto H, Kaguchi Y, et al. Association of Graves' disease with Evans' syndrome in a patient with IgA nephropathy. Intern Med. 2001. 40:1004–1010.

10. Geissler D, Ogriseg M, Fill H, Wolf H. Plasmapheresis treatment in 3 simultaneously occurring autoimmune diseases: Hashimoto thyroiditis with hyperthyroidism, autoimmune thrombopenia and autoimmune hemolytic anemia. Wien Klin Wochenschr. 1987. 99:351–355.

11. Hennemann HH, Kloss A. Autoimmune haemolytic anaemia, thrombocytopenia and thyroiditis: an immunopathological triad. Dtsch Med Wochenschr. 1978. 103:609–612.

12. Park MH, Kim HS, Kang SJ. HLA-A, -B, -DRB1 allele and haplotype frequencies in 510 Koreans. Tissue Antigens. 1999. 53:386–390.

13. Lio S, Albin M, Girelli G, Perrone MP, Gandolfo G, Conti L, et al. Abnormal thyroid function test results in patients with Fisher-Evans syndrome. J Endocrinol Invest. 1993. 16:163–167.

14. Aaltonen J, Bjorses P, Perheetupa J, Horelli-kuitunen N, Palotie A, Peltonen L, et al. An autoimmune disease, APECED, caused by mutations in a novel gene featuring two PHD-type zinc-finger domains. Nat Genet. 1997. 17:399–403.

15. Ide A, Eisenbarth GS. Genetic susceptibility in type 1 diabetes and its associated autoimmune disorders. Rev Endocr Metab Disord. 2003. 4:243–253.

16. Kie JH, Cho MS, Yang WI. Expression of CD40 and apoptosis related molecules in autoimmune thyroid diseases. Yonsei Med J. 2001. 42:488–496.