Angioimmunoblastic T cell lymphoma: pathobiological insights and clinical implications.

PURPOSE OF REVIEW: Angioimmunoblastic T cell lymphoma is a complex lymphoproliferative disorder. While recent evidence suggests that the Epstein-Barr virus and B cell disregulation are implicated in the disease's pathogenesis, their mechanistic roles remain largely unknown. The prognosis with traditional chemotherapy has been poor, but improved understanding of the disease's pathobiology has led to several promising novel therapeutic strategies.

RECENT FINDINGS: The recent finding of overexpression of the chemokine CXCL13 by the neoplastic cells of angioimmunoblastic T cell lymphoma suggests that it is derived from follicular helper T cells. In addition, gene-expression profiling has demonstrated overexpression of several genes characteristic of follicular helper T cells. Vascular endothelial growth factor-A is also highly expressed. Novel therapeutic strategies including immunomodulation with agents like cyclosporine and angiogenesis inhibition with drugs such as bevacizumab are being investigated, and show early promise in this disease.

SUMMARY: Diseases such as angioimmunoblastic T cell lymphoma can help illuminate the biology of the normal immune system. Significant progress has been made in understanding the biology of angioimmunoblastic T cell lymphoma. This has paved the way for the development of new therapeutic strategies and these have shown interesting results.