Immunoblastic lymphadenopathy: case report and literature review

A patient with immunoblastic lymphadenopathy (IL) had an unusual course of illness, with frequent episodes, over a 20-month period, of chills, fever, abdominal pain, hepatosplenomegaly and weight loss. The episodes were short-lived and many resolved spontaneously. Eventually generalized lymphadenopathy and profound monoclonal IgG gammopathy developed, with atypical mononuclear cells in the peripheral blood and increased numbers of plasmacytoid cells in the bone marrow. Lymph node biopsy revealed the morphologic triad typical of IL: proliferation immunoblasts, proliferation of small blood vessels and the deposit of an amorphous acidophillic material in the vascular walls and the interstitium. Up to October 1976 110 cases had been reported of this disorder, first described 3 years ago, which indicates that IL is not rare. Remissions have occurred spontaneously and after steroid therapy or chemotherapy or both. However, death has been reported in almost 50% of the cases, and the best approach to therapy remains to be determined.