Dublin 91: Joint Annual Scientific Meeting and Exhibition of the Royal College of Radiologists and the Faculty of Radiologists, the Royal College of Surgeons in Ireland. September 25-28, 1991, Dublin, Ireland, p. 135, 1991.. Unique Identifier : AIDSLINE ICDB/92686426 Bunn PA Jr; Dept. of Medicine, Div. of Medical Oncology, Univ. of Colorado; Cancer Center, Denver, CO
Abstract:
The Working Formulation classification of non-Hodgkin's lymphomas does not include most of the T-cell lymphomas. The cutaneous T-cell lymphomas are low-grade lymphomas, which include mycosis fungoides and the Sezary Syndrome. The malignant cells have a mature helper T-cell phenotype (CD2+, CD3+, CD4+, CD8-) and low expression of the IL2 receptor. They always have monoclonal rearrangement of the T-cell receptor beta chain gene. While cytogenetic abnormalities are frequent, no specific defect has been identified. These malignancies respond to the same therapies as other low-grade malignancies, including radiation, chemotherapeutic agents, interferons, fludarabine and deoxycoformycin. The best approach to treatment by stage is unknown. Combined modality treatment with whole-skin electron-beam irradiation and systemic combination chemotherapy was not found to be superior to a conservative approach with topical nitrogen mustard; UV A light irradiation with oral psoralen (PUVA), combined with sc injections of recombinant interferon alpha 2, gave the highest complete response rates reported to date. Adult T-cell leukemic/lymphoma (ATLL) is a high-grade lymphoma caused by the HTLV-1 virus. The cells also have a mature helper T-cell phenotype with T-cell receptor beta chain rearrangements, but have high expression of the IL-2 receptor. Patients have frequent opportunistic infections, bone involvement and hypercalcemia, and leptomeningeal involvement. Although response rates to intensive chemotherapeutic approaches are high, the survival is poor. New treatment approaches are needed. Peripheral T-cell lymphomas (PTL) may be intermediate- or high-grade and may be cured by current intensive combination regimens. Both ATLL and PTL may involve this skin, though not all patients have cutaneous involvement. Histologically, PTL can be distinguished from CTCL by the deep dermal involvement with sparing of the epidermis and upper dermis. ATLL may have dermal involvement, but occasionally has epidermal involvement as well. Histologically, PTL cannot be reliably distinguished from other diffuse large-cell, mixed-cell and immunoblastic lymphomas with a B-cell phenotype.
Keywords: Antigens, CD/ANALYSIS Human Interferons/THERAPEUTIC USE Leukemia, T-Cell, HTLV-II-Associated/GENETICS/*THERAPY Lymphoma, Non-Hodgkin's/GENETICS/IMMUNOLOGY/*THERAPY Lymphoma, T-Cell/GENETICS/IMMUNOLOGY/*THERAPY Pentostatin/THERAPEUTIC USE Skin Neoplasms/THERAPY Vidarabine/*ANALOGS & DERIVATIVES/THERAPEUTIC USE ABSTRACT 921230
M92C5364
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