Nephrotic syndrome associated with angiofollicular lymph node Hyperplasia

A woman presented at the age of 22 with anemia, hepatosplenomegaly, polyclonal hypergammaglobulinemia and a mediastinal shadow. At thoracotomy she had enlarged mediastinal lymph nodes which displayed histological features typical of angiofollicular hyperplasia. Marrow plasmacytosis was present. She developed diabetes mellitus at the age of 29 yrs after she had received oral steroid treatment for one year. The nephrotic syndrome supervened another year later. Her kidneys were enlarged before the onset of diabetes. The glomerular changes included a marked increase of mesangial matrix and segmental hypercellularity. The association of the nephrotic syndrome and angiofollicular lymph node hyperplasia is extremely rare and their interrelation remains enigmatic.