Rosai-Dorfman-Destombes disease in an elderly man. A case of necrosis in the lymph node

Rosai-Dorfman-Destombes disease (RDD), often associated with autoimmune disease, is rare in the elderly. We report a 79-year-old Japanese man with RDD, who had been treated for non-specific interstitial pneumonia (NSIP) for longer than 6 years.

Detecting RDD lesions was evaluated with imaging studies, including PET-CT, and the RDD diagnosis was based on the pathological findings of a biopsied lymph node. Findings and Clinical course: PET-CT revealed an FDG-avid 5.5 cm-sized mass (SUVmax; 7.12) at the right lung and enlarged lymph nodes at the bilateral supra-clavicular area (SUVmax; 10.12). A supraclavicular lymph node biopsy confirmed the diagnosis of RDD; however, it was characterized by lymph node necrosis, which is rarely noted in the RDD tissue. Three months after the RDD diagnosis, the patient developed cold agglutinin disease (CAD), causing severe anemia, for which packed red blood cell transfusions and sutimlimab^® therapy were planned; however, the patient died of presumable NSIP exacerbation.

RDD occurs even in the elderly. RDD in this case was associated with autoimmune NSIP and CAD. The presence of necrotic foci in the biopsied lymph node does not contradict the diagnosis of RDD.