Rosai-Dorfman Disease (RDD) is characterized by histiocytic proliferation in the sinuses of
affected mediastinal lymph nodes. We report a patient with fever of unknown origin (FUO) who
was treated for secondary thrombotic microangiopathy (TMA);however, postmortem histology
revealed RDD. TMA was treated with 12 rounds of plasma-exchange therapy, but this was only
partially effective. A hematological tumor (Castlemanʼs disease) was suspected and intravenous
pulsed steroids and oral prednisolone therapy improved the patientʼ s general condition.
Unfortunately, the patient died due to acute myocardial infarction. Postmortem analysis of
paratracheal lymph adenopathy nodes revealed the proliferation of histiocytes exhibiting
emperipolesis, characteristic of RDD.
A 50-year-old man was referred to us due to palpitation, sweating, and body weight loss.
He had an enlarged thyroid with hyperthyroidism and was positive for anti-TSH receptor antibody, leading to diagnosis of Gravesʼ disease. He also showed an enlarged parotid, and
cytology revealed myoepithelial cells with interstitial mucus, which was diagnostic for pleomorphic parotid adenoma.