Korean J Hematol.  2011 Dec;46(4):279-282. 10.5045/kjh.2011.46.4.279.

Evans syndrome following long-standing Hashimoto's thyroiditis and successful treatment with rituximab

• Oh HJ
• Yun MJ
• Lee ST
• Lee SJ
• Oh SY
• Sohn I

• KMID: 2251990
• DOI: http://doi.org/10.5045/kjh.2011.46.4.279

Abstract

We report a case of a 51-year-old woman with Evans syndrome (autoimmune hemolytic anemia and primary immune thrombocytopenia) and hypothyroidism. She was previously diagnosed with Hashimoto's thyroiditis in 1994 (age, 35) and autoimmune hemolytic anemia (AIHA) 3 years ago. She was treated with oral prednisolone. After a period, in which the anemia waxed and waned, there was an abrupt development of thrombocytopenia (nadir 15x10(9)/L) that coincided with the tapering off of prednisolone after 3 years of administration. Because her thrombocytopenia was refractory to prednisolone, we administered rituximab (375 mg/m2 weekly) for 4 weeks. Two weeks after the completion of the rituximab treatment, her platelet count was up to 92x10(9)/L. No intermittent peaking of thyroid stimulating hormone occurred after rituximab treatment was initiated. Evans syndrome and autoimmune thyroiditis might share common pathophysiological mechanisms. This notion supports the use of rituximab in a patient suffering from these disorders.

Keyword

Evans syndrome; ITP; AIHA; Autoimmune thyroiditis

MeSH Terms

Anemia
Anemia, Hemolytic
Anemia, Hemolytic, Autoimmune
Antibodies, Monoclonal, Murine-Derived
Female
Humans
Hypothyroidism
Middle Aged
Platelet Count
Prednisolone
Stress, Psychological
Thrombocytopenia
Thyroid Gland
Thyroiditis
Thyroiditis, Autoimmune
Thyrotropin
Rituximab
Antibodies, Monoclonal, Murine-Derived
Prednisolone
Thyrotropin

Figure

• 

  Fig. 1 Trends in Hb level and platelet count with PD dosage and rituximab treatment. PD indicates dose of prednisolone. The units of Hb and PLT in this figure are g/dL and ×104/µL, respectively. Date of diagnosis of diseases and treatment of rituximab is expressed at the bottom of figure.Increase in dose of PD elevated Hb level but not PLT level. PLT level is increased after the use of rituximab.

• 

  Fig. 2 Relationship between TSH level and doses of L-thyroxine and rituximab. The unit of L-thyroxin and TSH is ×10-2 mg and ×10-2 mIU/L, respectively, in this figure. After rituximab treatment, there is no increase in TSH level and the required dose of L-thyroxin is decreased.

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