Clinicopathologic and therapeutic aspects of angioimmunoblastic lymphadenopathy‐related lesions

Abstract

The clinicopathologic features of 14 patients with angioimmunoblastic lymphadenopathy (AIL)‐related lesions were analyzed. Lymph node biopsy specimens from all the patients showed a diffuse obliteration of lymph node architecture, prominent vascular proliferation, a polymorphous cellular infiltrate, including immuno‐blasts, and varying degrees of clear cell proliferation. The patients were eight males and six females, with a median age of 58.5 years. All but one were in an advanced stage at the time of diagnosis. Bone marrow involvement was observed in eight patients. Thirteen patients had a negative serologic reaction for antibody to human T‐cell leukemia virus type I (HTLV‐I), and one patient was considered to be a HTLV‐I carrier. Polyclonal hypergammaglobulinemia was observed in 6 patients, and 6 of the 12 patients showed elevated IgE levels. Immunophenotyping of the involved lymph nodes revealed a preponderance of T‐cells in all the patients. Eleven of these patients showed a predominance of CD4⁺ over CD8⁺ T‐cells, and only one patient showed a predominance of CD8⁺ over CD4⁺ T‐cells. Two of five patients whose gene analysis was carried out showed clonal rearrangement of the T‐cell receptor beta chain gene without rearrangement of the immunoglobulin heavy chain genes. Twelve patients received doxorubicin‐containing combination chemo therapy; of these, 7 patients achieved complete response, and the other 5 had partial response. Nine patients are still alive with a median follow‐up period of 21 months, and five patients died during the follow‐up period. Progression to high‐grade T‐cell lymphoma with systemic infiltration was ascertained in two of three cases for which autopsy was performed. From our experience, we recommend doxorubicin‐containing combination chemotherapy as initial therapy for AIL‐related lesions.