Acute renal failure in a patient with Rosai-Dorfman disease

Acute renal failure developed in a 57-year-old woman who had Rosai-Dorfman disease diagnosed 1 year previously on a cervical lymph node. Organ imaging showed diffuse masses infiltrating both kidneys. The renal biopsy showed a lymphoplasmacytic and histiocytic process extensively replacing the parenc hyma, which is in keeping with Rosai-Dorfman disease of the kidneys. Howeve r, the typical lymphophagocytic cells were lacking. This case illustrates t hat diagnosis of Rosai-Dorfman disease in renal biopsy can be very difficul t, requiring both exclusion of many benign and malignant lesions and a high index of suspicion for this condition. In particular, lymphoma was exclude d based on the mixed polyclonal composition of inflammatory cells and the a bsence of atypical lymphoid proliferation. The renal function partially rec overed after a course of therapy combining VP-16 (etoposide) and dexamethas one and remained stable over 4-year follow-up. This report emphasizes the i mportance of early diagnosis and intervention to safeguard renal function i n extensive Rosai-Dorfman disease. (C) 1999 by the National Kidney Foundati on, Inc.