Serum Permeability Activity in Steroid-Resistant Minimal Change Nephrotic Syndrome Is Abolished by Treatment of Hodgkin Disease

A circulating permeability factor is present in some patients with minimal change nephrotic syndrome (MCNS) or focal segmental glomerulosclerosis. Nephrotic syndrome occurs in less than 1% of patients with Hodgkin disease. A substance derived from T lymphocytes may be responsible for proteinuria in these patients, but a circulating permeability factor was not shown. Serum permeability activity (P_alb) of a young man who presented with MCNS was tested over 11 years. He first was treated with oral prednisone, then cyclosporine (CsA; 4 mg/kg/d). Two years after the initial diagnosis, during CsA-induced remission of nephrotic syndrome, Hodgkin disease was diagnosed and he underwent systemic chemotherapy with doxorubicin hydrochloride, bleomycin, vinblastine, and dacarbazine and radiation therapy. P_alb was 0.67 before CsA therapy. Although CsA treatment decreased proteinuria to protein less than 100 mg/d, P_alb did not change. P_alb decreased to 0.19 within 2 weeks of initiation of chemotherapy for Hodgkin disease and has remained at less than 0.17 for the last 9 years. The patient, in remission from Hodgkin disease, has normal renal function and no detectable proteinuria. This is the first demonstration of the presence of P_alb in a patient with MCNS and subsequent Hodgkin disease. It also is the first report that aggressive chemotherapy abolishes P_alb. Although the potential causal relationship between nephrotic syndrome and Hodgkin disease in this patient is not clear, the immediate decrease in P_alb during treatment suggests that aggressive chemotherapy may be an effective treatment for patients with high P_alb in steroid-resistant MCNS or focal segmental glomerulosclerosis.