Abstract
A 17-year-old male was admitted because of severe anemia and polyclonal hypergammaglobulinemia and diagnosed as idiopathic plasmacytic lymphadopathy with polyclonal hyperimmunoglobulinemia (IPL).
IPL is a distinct new clinical entitiy of plasma cell dyscrasias, proposed by Mori et al in 19801). This disease is clinically characterized by systemic lymphadenopathy and marked polyclonal hypergammaglobulinemia and pathologically by specific histological findings of the lymphnodes including follicular hyperplasia and remarkable interfollicular mature plasmacytosis without evidence of malignant growth.
This 17 year old patient was the youngest in age and had the highest serum gammaglobulin level among previously published cases of IPL, and therapy with anticancer drugs was vequired because of hyperviscosity syndrome resulting from hypergammaglobulinemia and concomitant severe anemia.
In this report we describe the effectiveness of melphalan which seemed superior to other drugs judged from his clinical course. We also discuss the distinction of IPL from plasma cell types of giant lymphnode hyperplasia and from the other diseases that are similar to IPL but different hith regard to the sites of involvement which include the skin or lung.
