Intravascular malignant lymphomatosis involving the kidney: three case reports

Abstract

We report three autopsy cases of patients with renal symptoms related to intravascular malignant lymphomatosis (IML) exclusively involving both kidneys. The patients were one woman and two men, aged 64, 65, and 82 years, respectively. Their presenting symptoms included fever, anemia, thrombocytopenia, icterus, dyspnea, weight loss, and enlarged adrenal glands. All patients presented with acute renal dysfunction, with creatinine ranging from 2.0 to 6.5 mg/dl, or with modest proteinuria. One patient had an underlying bilateral disease, chronic pyelonephritis. Another patient also had an autoimmune disease. None of the three patients were aggressively treated. All died of progressive disease 2 weeks to 2 months after the onset of their renal symptoms. The diagnosis of the lymphomas was confirmed by morphological and immunohistochemical studies of the postmortem material. The lymphomas were subclassified as large-cell, characterized by angiotropism. Immunophenotyping revealed that all were B-lineage neoplasms. Renal involvement by IML was characterized histologically by a unique pattern of multifocal neoplastic disseminations within the glomerular and peritubular capillaries, with relative sparing of the interstitum. In one patient, glomerular mesangiolysis in association with embolic proliferations of lymphoma cells was a striking feature. All patients had bilateral renal involvement by IML which occurred in a setting of widespread disease. Our studies indicate that IML is an unusual subset of secondary renal lymphomas with bilateral diseases, and, rarely, may produce renal symptomatology early in the course.