Good syndrome and polymyositis

摘要

Good syndrome (GS) was first described by Dr Robert Good in 1954 in an adult patient with thymoma and immunodeficiency. The immunodeficiency found in GS typically demonstrates low to absent B cells in the peripheral blood, hypogammaglobulinemia, and defects in cell-mediated immunity in patients 40 to 70 years of age. Infections associated with both B- and T-cell defects are common. Autoimmune disorders and their complications have been associated with GS. Although dysregulation of T-cell function has been implicated, the pathogenesis remains unclear. In a systematic review by Kelesidis and Yang of 152 patients diagnosed as having GS, pure red blood cell aplasia and myasthenia gravis were the most prevalent comorbidities, followed by oral lichen planus, aplastic anemia, macrocytic anemia, autoimmune hemolytic anemia, monoclonal gammopathy, diabetes mellitus, polyarthropathy, and myelodysplastic syndrome. Polymyositis has never been reported in a patient with GS.