Rosai-Dorfman disease with paratracheal adenopathy presenting as a fever of unknown origin (FUO)

Abstract

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.