Angioimmunoblastic lymphadenopathy-type peripheral T-cell lymphoma with cutaneous infiltration: report of a case and its gene expression profile

Angioimmunoblastic T-cell lymphoma is a type of peripheral. T-cell lymphoma that is clinically characterized by high fever and generalized lymphadenop athy with or without cutaneous involvement. A 55-year-old Japanese man pres ented with red papular lesions on the trunk and limbs, oedema, and generali zed lymphadenopathy, Histological findings in the lymph nodes showed destru ctive germinal centres, proliferation of arborizing postcapillary venules, and atypical medium-sized lymphocytes, The cutaneous lesions also contained atypical lymphocytes. Immunohistochemical studies indicated that the neopl astic cells were mature CD4+ T lymphocytes. Southern blot analysis detected a clonal expansion of T-cell receptor beta. Based on these findings, a dia gnosis of angioimmunoblastic T-cell lymphoma with cutaneous infiltration wa s made. Despite systemic chemotherapy, the disease exhibited a high level o f activity and continued on a fatal course, An analysis of gene expression profiling using complementary DNA microarrays revealed significant expressi on of some chemokines and cytokines, e.g. secondary lymphoid tissue chemoki ne, macrophage inflammatory protein (MIP)-1 beta, MIP-3 alpha, MIP-3 beta, B-lymphocyte chemokine, interleukin-16 and tumour necrosis factor-beta, and an apoptosis-inhibitory protein (FLICE inhibitory protein) in the affected lymph nodes. Profiling of gene expression patterns for a variety of genes in additional cases may be helpful in determining which factors predict the biological and clinical behaviour of angioimmunoblastic T-cell lymphoma or other aggressive malignant lymphomas.