Angioimmunoblastic lymphadenopathy with dysproteinemia in Thailand

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a disease of unknown etiology and pathogenesis. It has the features of hyperimmunity and immune deficiency, and its behavior resembles malignant lymphoma. We report a review of 16 patients with AILD seen at Ramathibodi Hospital from 1982 to 1986. Thirteen patients had fever and seven had pruritus and rashes. Lymphadenopathy was found in all 16 cases; generalized in 14 and localized in 2. Hepatomegaly was present in 14 patients while only 7 had splenomegaly. Laboratory findings included autoimmune hemolytic anemia, lymphocytosis and polyclonal hypergammaglobulinemia. Pulmonary involvement was seen in 5 cases, and bone marrow showed the characteristic features of the disease in 9 cases. Two patients went on to develop diffuse lymphocytic, poorly differentiated lymphoma. Fourteen patients were treated with prednisolone initially. Five responded with complete recovery, eight responded with partial recovery, and one died with extensive involvement of the disease. Six of the patients that recovered partially were later treated with cyclophosphamide, vincristine and prednisolone. One patient recovered completely and two partially. Three died from extensive involvement. Two patients with malignant lymphoma were treated by combination chemotherapy. One case went to complete remission while the other died from infection. One patient was lost to follow up before any treatment was started.