Autoimmune hemolytic anemia in a patient with Kabuki syndrome - a case report in São Paulo and a brief discussion about Kabuki syndrome and association with autoimmunity

Kabuki syndrome (KS), first described in Japan in 1981, is a rare disease characterized by a peculiar facies, postnatal growth deficiency, intellectual disability and a set of malformations, such as skeletal and visceral abnormalities. In the natural history of KS, abnormal immune events are presents and this case report contribute with the understanding and consolidation of hematologic autoimmune disorders. This article describes a case report of a patient with previous diagnosis of Kabuki syndrome with an autoimmune hemolytic anemia in a hospital of São Paulo, with adequate response and resolution with corticosteroids therapy. The article also makes a brief explanation of correlation with autoimmunity, immunodeficiency and Kabuki syndrome.