Nephrectomy was performed for uncontrollable unilateral hematuria in an app
arently healthy 72-year-old man. The suburothelial connective tissue of the
kidney was infiltrated by primitive myeloid cells with associated acute va
sculitis and foci of extramedullary hematopoiesis. Subsequently, the patien
t was shown to have chronic myelomonocytic leukemia. Although renal involve
ment and vasculitis have been recorded previously in chronic myelomonocytic
leukemia, this is the first occasion, to our knowledge, where their concur
rence resulted in such a spectacular presentation.