Classic Hodgkin lymphoma in a patient with Behçet’s disease: a case report and review of the literature

Behçet’s disease (BD) is a chronic relapsing inflammatory vasculitis associated with an increased risk of malignancy, particularly hematologic cancers. However, reports of Hodgkin lymphoma (HL) in BD remain rare.

A 21-year-old female with BD on long-term glucocorticoid therapy who presented with fatigue, dyspnea, and oral ulcerations in the setting of an enlarging anterior mediastinal mass. Laboratory evaluation revealed microcytic anemia, leukocytosis, thrombocytosis, and elevated inflammatory markers. Infectious and autoimmune evaluations were unrevealing for other etiologies. Biopsy of the mediastinal mass confirmed Epstein-Barr virus (EBV)-positive classic HL, and PET-CT demonstrated FDG-avid nodal involvement. The patient was discharged with oncology follow-up for planned chemoradiation.

BD and HL are shown to have minimal associations in the literature, and thus, this review illustrates a rare but important association between BD and HL, possibly mediated by immune dysregulation and chronic immunosuppression. Clinicians should maintain a high index of suspicion for malignancy in BD patients on long-term steroids presenting with atypical or progressive findings, such as a mediastinal mass, to enable prompt diagnosis and treatment.