Angioimmunoblastic lymphadenopathy with retinitis and drug related exacerbations: a clinicopathological case study

A patient with angioimmunoblastic lymphadenopathy with dysproteinemia was followed over a three year period from diagnosis to death. He presented with arthralgias, uveitis and respiratory insufficiency and developed hyperuricemic renal failure upon institution of treatment. Aggressive combination chemotherapy was required to reverse progressive thrombocytopenia and pulmonary involvement. A complete remission was achieved twice. There was a striking temporal relationship between the administration of antibiotics or allopurinol and exacerbations of the disease. Hypocomplementemia and transient evidence of vasculitis suggested the presence of immunecomplexes. Serial lymph node biopsies showed the progression of this disorder from a pleomorphic immunoblastic proliferation to a lymphocyte-depleted, fibrotic process, in parallel with a decline from hyper- to hypogammaglobulinemia. This case illustrates the broad clinical spectrum of angioimmunoblastic lymphadenopathy with dysproteinemia and suggests that aggressive treatment is necessary in selected patients.