Autoimmunity in a patient with 47,XXX karyotype

Premature ovarian failure in women with a 47,XXX karyotype have been described, but an explanation for this gonadal disorder has not been forthcoming. The present case identifies a 47 triple X woman with premature ovarian failure associated with an autoimmune disorder. A possible association between the 47,XXX karyotype, autoimmune disorders, and premature ovarian failure is proposed.