Abstract
The association between Hodgkin Disease (HD) and paraneoplastic Nephrotic Syndrome is well documented in adults but is relatively uncommon in the pediatric population. We describe two children with HD who initially presented with Nephrotic Syndrome. Case 1: 12- year-old boy who presented with a two-week history of periorbital edema, proteinuria and hypertension and was diagnosed with Nephrotic Syndrome. The renal biopsy showed minimal change disease. Since the patient developed dyspnea, further work-up was done and he was found to have a large mediastinal mass. Core needle biopsy of the mass was consistent with HD, nodular sclerosing type. Case 2: 14-year-old boy diagnosed to have Nephrotic Syndrome with biopsy proven minimal change disease and was treated intermittently with steroids over 6 months. He subsequently developed dyspnea and was found to have a right paratracheal mass and excisinal biopsy was consistent with HD, nodular sclerosing type. Both these patients initially presented with Nephrotic Syndrome which completely resolved after the first course of chemotherapy for HD. Upon review of literature, there have been 23 pediatric cases of Nephrotic Syndrome associated with HD in patients ranging in age between 2–15 years. The renal biopsy was not done for 8 of these patients and the diagnosis was established clinically. Fifteen patients had biopsies, 9 had minimal change disease, 1 mesangial hypercellularity, 1 glomerulosclerosis, 1 undetermined histology and 3 were normal. In all cases, the Nephrotic Syndrome was refractory to steroids and resolved with treatment of the HD. Pathogenesis of the Nephrotic Syndrome associated with HD is not known, however there are speculations about T-cell involvement and production of lymphokines and humoral factors which may be responsible for the glomerular damage.
NEPHROTIC SYNDROME AS PARANEOPLASTIC SYNDROME IN A PATIENT WITH THYMIC MASS
