Management of a young patient with combined autoimmunity: Evans Syndrome: a case report Andres Pinto, DMD, a Rochelle G. Lindemeyer, DMD, b and Faizan Alawi, DDS, c Philadelphia, PA UNIVERSITY OF PENNSYLVANIA SCHOOL OF DENTAL MEDICINE 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. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:505-11) Evans syndrome (ES) is defined by the simultaneous presentation of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). 1,2 These conditions may appear concomitantly or one may pre- cede the other by several months. The syndrome was described by Evans et al. in 1949 and 1951. 2 His original report included 24 patients with varied clinical presentation, including hemolytic anemia and thrombo- cytopenia. Although Evans’ report constituted the ini- tial documentation of ES, 15 patients from his sample had either AIHA or ITP only. Given the dramatic response to splenectomy, Evans suggested that these disorders shared a similar etiology. Subsequent reports have followed several cohorts over time, and most adult cases have been linked to other diseases, such as sys- temic lupus erythematosus and aplastic anemia. 3-5 Dis- covery of the etiology of ES in pediatric patients has been elusive, because children with this clinical presen- tation are often evaluated for other autoimmune disease or multisystemic involvement. 6,7 Dental management of patients diagnosed with ES requires knowledge and familiarity with the clinical manifestations and thera- peutic approach to this syndrome. We present the first reported case, to our knowledge, of the dental manage- ment of a young individual diagnosed with ES. 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 Phil- adelphia, 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 an- tibiotic prophylaxis diagnosed in 2002. She had been hospi- talized multiple times for hemorrhagic episodes requiring blood transfusions during the preceding 5 years. At the time of presentation, she had been asymptomatic for 10 months and was closely monitored with serial bimonthly hematologic screening. She denied any surgical history or allergies to medications and was currently only taking over-the-counter analgesics for dental pain. A detailed review of systems revealed intermittent dyspnea on exertion, easy bruising (oral mucosa and skin, in association with cytopenic crisis), and generalized articular and skeletal pain. Her family history was noncontributory. She denied any alcohol or tobacco use. Upon physical examination, we found a well nourished, well developed young female in no apparent distress. Her vital signs were within normal limits, and she did not have any head and neck asymmetry, swelling, or lymphadenopathy (Fig. 1). Intraoral exam revealed multiple carious teeth, pos- terior bite collapse, anterior open bite, posterior cross bite, dental malposition, notched maxillary anterior incisors, and no soft tissue pathology, vestibular swelling, or signs of mucosal bleeding (Figs. 2 and 3). The patient had refused dental care in the past owing to fear of bleeding and surgery. Laboratory values at presentation are seen in Table I. Inter- estingly, ES in this patient presented with episodes of throm- bocytopenia and intermittent neutropenia. The clinical ex- pression of AIHA was not as prominent, although she did complain of frequent shortness of breath. Owing to severe carious involvement, 11 teeth were indi- cated for extraction. The preliminary treatment plan included a Hawley appliance to open the vertical dimension of occlu- sion, operative dentistry, and fabrication of removable pros- theses to restore function. The patient and her mother were a Assistant Professor, Oral Medicine. b Assistant Professor and Residency Program Director, Pediatric Den- tistry. c Assistant Professor, Pathology. Received for publication Jun 14, 2006; returned for revision Jul 19, 2006; accepted for publication Jul 25, 2006. 1079-2104/$ - see front matter © 2007 Mosby, Inc. All rights reserved. doi:10.1016/j.tripleo.2006.07.022 505