Abstract / Summary
Evans syndrome is a rare autoimmune disorder characterized by immune-mediated cytopenias and chronic immune dysregulation. Its association with giant cell lesions of the jaws is extremely uncommon. A 52-year-old woman with Evans syndrome and a history of mandibular CGCG and maxillary PGCG presented with multiple mandibular giant cell lesions in 2024, with CGCG histopathologically confirmed in the right posterior mandibular and left parasymphyseal lesions. Treatment was individualised according to lesion size, location, and relationship with adjacent anatomical structures. The right posterior mandibular lesion involved a large portion of the atrophic mandible and was closely related to the inferior alveolar nerve. Because surgical treatment was considered to carry a risk of pathological fracture and neurosensory injury, intralesional corticosteroid therapy was administered. In contrast, the smaller and more accessible left parasymphyseal lesion was treated by surgical excision/curettage without adjuvant corticosteroid therapy. The unbiopsied lesions in the left mandibular body and angle were managed with clinical and radiographic follow-up. At the 1-year CBCT follow-up, the approximate dimensional product increased for the right posterior mandibular and left mandibular angle lesions, decreased for the surgically treated left parasymphyseal lesion, and remained stable for the left mandibular body lesion. The coexistence of Evans syndrome and giant cell lesions of the jaws highlights the importance of considering systemic conditions when evaluating these lesions. An integrated clinical, radiographic, histopathological, and systemic assessment may support diagnosis and individualised management.