ANGIOIMMUNOBLASTIC LYMPHADENOPATHY WITH DYSPROTEINEMIA - EMPHASIS ON PATHOGENESIS AND TREATMENT

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rar e lymphoproliferative disorder characterized by diffuse lymphadenopath y, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hyperga mmaglobulinemia. Morphologically, the involved lymph nodes demonstrate complete effacement of the normal architecture, prominent neovascular ization and infiltration by immunoblasts and plasma cells. Other terms that have been used to describe this entity include diffuse plasmacyt ic sarcomatosis, immunoblastic lymphadenopathy, lymphogranulomatosis X , and immunologic aberrations in idiopathic reticulosis. Initially, AI LD was thought to be a disease of B-cell origin that represented react ive immune response to unknown stimulus and high potential for maligna nt transformation. It is now evident that AILD in 80% of cases follows an aggressive course with short median survival, especially, if compl ete response with chemotherapy is not achieved. Immunologic and molecu lar studies have demonstrated that the majority of AILD cases are T-ce ll clonal disorders. Despite the numerous reports on the role of Epste in-Barr virus in this disorder, it is unknown whether the presence of this virus is associated with the immune defect that accompanies AILD, or whether it is a pathogenetic factor. In contrast to non-Hodgkin's lymphomas, a stage is not usually assigned to the patient since the di sease is systemic in nature, subsequently, parameters such as extent o f disease and tumor bulk used to identify high-risk patients with non- Hodgkin's lymphomas, do not appear to correlate with disease activity or prognosis in AILD. Treatment of AILD has been unsatisfactory, with approximately 25% of patients achieving complete and sustained remissi on when combined chemotherapy agents are used. This article is devoted to a discussion of the different manifestations, suggested pathogenes is, and treatment of AILD.