Oral medicine
Management of a young patient with combined autoimmunity: Evans Syndrome: a case report

https://doi.org/10.1016/j.tripleo.2006.07.022Get rights and content
Management of patients with autoimmune disease can present a challenge for clinicians. Combinations of autoimmune disorders carry a worse prognosis and are often difficult to diagnose. Evans syndrome is a rare hematologic disorder characterized by the concurrent presentation of autoimmune hemolytic anemia and immune thrombocytopenic purpura. This paper reports the first case of the dental management of a young patient with Evans, discusses the current literature surrounding the differential diagnosis and treatment of Evans syndrome, and suggests a guide for the oral medicine, pediatric dentist, or hospital dental clinician when treating these patients.

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Case Report

A 17-year-old African American female was referred to the University of Pennsylvania, School of Dental Medicine, Oral Medicine Clinic, from the Children’s Hospital of Philadelphia, for dental evaluation. The patient was diagnosed with ES at the age of 3. Her medical history was otherwise significant for a physiologic heart murmur not requiring antibiotic prophylaxis diagnosed in 2002. She had been hospitalized multiple times for hemorrhagic episodes requiring blood transfusions during the

Discussion

Evans syndrome was initially reported by Fisher8 and Evans.2 The combination of AIHA and ITP can herald the development of autoimmune disease, systemic connective tissue disease, lymphoproliferative disorders, and neutropenia.9 The median age of presentation of this condition ranges between 5.5 and 7.7 years, with a wide age range reported in the literature. Uncommon in infants, the onset of ES in patients younger than 12 months carries a somber prognosis and is characterized by resistance to

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