Neoplastic PTLD. Case 3.
Large cell (monomorphic) lymphomatous PTLD involving the kidney
This young woman developed a PTLD which presented as flank pain 6 years after liver transplantation. Immunosuppression consisted of Cyclosporine A, azathioprine, and prednisone. She had also received anti-lymphocyte globulin in the past. The kidney contained a 16 cm tumor with a uniform mononuclear population of cells. The original diagnosis was diffuse large cell non-Hodgkins lymphoma. In the original Pittsburgh terminology this would be termed a monomorphic PTLD. It would now be considered lymphomatous (neoplastic) PTLD, diffuse large cell type. Both immunophenotypic studies and immunoglobulin gene rearrangement studies disclosed a monoclonal population of cells. EBV was demonstrated within the tumor cells as well. The tumor extended into the perirenal tissue and also was seen in a cervical lymph node biopsied at the same time. Following surgical resection, immunosuppression was reduced and no other therapy was given. Four months later, she developed hemorrhagic pancreatitis and sepsis. She became hypotensive and expired. At autopsy, residual PTLD was found in hepatic hilar nodes. However, no disease was seen elsewhere and this was not considered to be a factor in this patients death. There was no other evidence of tumor following the original surgery. At the time of the development of pancreatitis, it was thought that this might reflect recurrence of tumor. However, this was shown not to be the case. The tempo of changes in the hepatic hilar node PTLD cannot be known with certainty. The nodes were necrotic. This may reflect an element of regression, which may occur over a period of several months. Since the nodes were first encountered at autopsy, it could also be argued that this represents a recurrence of disease.
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