Lenner's lymphoma C84.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

atypical lymphoepitheloid cell proliferation; epitheliodcellular lymphogranulomatosis; Lymphoepitheloid cell proliferation atypical; lymphoepitheloid lymphoma; Lymphoma lymphoepitheloides; Proliferation lymphoepitheloid cell

History
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Lennert and Mestdagh, 1968

Definition
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Epithelial cell-rich, small cell peripheral cutaneous T-cell lymphoma, which is very close to lymphogranulomatosis. Occasionally, specific infiltrates of the skin were observed.

Clinical features
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  • Skin lesions: truncated, mostly follicularly bound, small, skin-coloured to red, rough papules, which can conglomerate to larger plaques with a shiny surface. Scarring alopecia, ulcerations (especially genital).
  • Extracutaneous manifestations: mediastinal, hiliary lymph node enlargement and intrapulmonary round foci.

Histology
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In lymph nodes, bone marrow and affected skin, T-cell infiltrate rich in epithelial cells is present. Focally, histiocytes, epitheloid cells and UCHL1-positive lymphocytes are present.

Therapy
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Polychemotherapy (e.g. COPP and/or ABVD scheme), radiotherapy, excision of single foci.

Literature
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  1. Kim H, Jacobs C et al (1978) Malignant lymphoma with a high content of epitheloid histiocytes. Cancer 41: 620-635
  2. Kojima H et al (2003) Rapidly progressive Lennert's lymphoma terminating in fulminant hepatic failure. Leuk lymphoma 44: 1409-1412
  3. Lennert K, Mestdagh J (1968) Lymphogranulomatoses with constantly high epithelial cell content. Virchows Arch Path Anat 344: 1-20
  4. Roundtree JM, Burgdorf WHC et al (1980) Cutaneous involvement in Lennert's lymphoma. Arch. Dermatol. 116: 1291-1294
  5. Uppenkamp M et al (2002) Classification of malignant lymphoma. Oncology 25: 563-570

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Last updated on: 29.10.2020