Peripheral T- cell lymphomas are an extremely heterogeneous group of disorders in which the histological, immunological, and clinical features are dictated by the mix of cytokines generated by the malignant cells, which may themselves be a relatively small constituent of a tumour. Often very confusing! PTCL is a rare group of non-Hodgkin's lymphomas derived from neoplastic peripheral (not central) T lymphocytes. There is a wide spectrum of clinical and histologic presentations amongst patients with these lymphomas. PTCL may involve lymphoid tissue anywhere. Systemic symptoms (B symptoms) are common and often severe. The diagnosis is often difficult due to: a) the diverse properties of the malignant clone in terms of cell surface receptors and the t-cell products released from it, which may encompass the entire T cell repertoir b) lesions often contain an abundance of non-T cells. c) mature T cell markers may be absent d) it may be difficult to demonstrate clonality The prognosis is poor, and while the lesions are usually initially responsive to treatment, relapse is early and multi-drug resistance readily occurs.
Author(s): Peter R. Galbraith MD
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