[Systemic amyloidosis associated with common variable hypogammaglobulinemia and intestinal lymphoid nodular hyperplasia]

Abstract

Common variable hypogammaglobulinemia syndrome with lymphoid nodular hyperplasia of the intestine forms part of the so-called hypogammaglobulinemic enteropathies. It is characterized by decreased serum immunoglobulins, recurrent respiratory tract infections and chronic diarrhea. The development of systemic amyloidosis is infrequent, but it can be explained by the multiple infections in this setting. The case of a 47-years old woman with hypogammaglobulinemic enteropathy, who developed systemic amyloidosis is presented. It was manifested as a nephrotic syndrome. The previously published reports include 12 cases of common variable hypogammaglobulinemia with systemic amyloidosis. Half of them presented nephrotic syndrome as a manifestation of their amyloidosis. It is important to keep in mind this complication in these patients' follow-up in order to increase the doses of gammaglobulin. That is the way to compensate their additional losses because of the nephrotic syndrome that they usually develop.