RAPIDLY PROGRESSIVE T-CELL LYMPHOMA PRESENTING AS ACUTE-RENAL-FAILURE- CASE-REPORT AND REVIEW OF THE LITERATURE

We describe a case of peripheral T cell lymphoma that is remarkable fr o its fulminate course and selective targeting of both kidneys. The pa tient was a 6-year-old girl who was in her usual state of good health until the onset of abdominal pain and fever. She was treated for acute oliguric renal failure and visual disturbances. A renal biopsy was pe rformed. Biopsy findings were interpreted as suggestive of a vasculiti c process, and treatment was initiated for a presumptive diagnosis of Wegener's granulomatosis. The patient died 3 days following admission, and autopsy revealed extensive bilateral kidney infiltration by a per ipheral T cell lymphoma. The remainder of the body was spared with the exception of mild infiltration of the pulmonary parenchyma and choroi d plexus by neoplastic lymphocytes. The neoplastic nature of the disea se was confirmed utilizing immunoperoxidase stains and T cell receptor gene rearrangement. Primary renal lymphoma and renal failure attribut able to involvement by lymphoma are rare findings that should be consi dered when other more common causes of renal insufficiency have been e xcluded. The presenting clinical complaints are generally of short dur ation, nonspecific, and atypical. Most patients exhibit oliguria. Phys ical examination may reveal hepatosplenomegaly, lymphadenopathy, and f lank and/or abdominal mass(es). Laboratory findings frequently include an elevated serum creatinine, blood urea nitrogen, lactate dehydrogen ase, and a mild proteinuria. Electrolyte abnormalities are variably pr esent. Possible radiographic findings include hypodense or hypoechoic renal lesions and diffuse bilateral renal enlargement. Although the pr ognosis is dismal, survival may be prolonged utilizing current treatme nt modalities, and rare patients may be ''cured'' of disease. The clin ical presentation, radiological findings, and prognosis of patients wi th clinically evident renal involvement by non-Hodgkin's lymphoma are discussed.