Lymphoma cutaneous t-cell lymphoma pleomorphic small-medium- large-cell C84.4

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Synonym(s)

Cutaneous pleomorphic small-medium large cell T-cell lymphoma; Pleomorphic cutaneous T-cell lymphoma; Pleomorphic small-medium- large-cell cutaneous T-cell lymphoma; Pleomorphic small-medium- large-cell T-cell lymphoma of the skin; Pleomorphic T-cell lymphoma of the skin

Definition
This section has been translated automatically.

Pleomorphic T-cell lymphomas of the skin can be differentiated according to histological criteria into small, medium and large-cell variants, whereby the large-cell variants are in turn differentiated into a CD30-positive and a CD30-negative form (different prognosis, see below lymphoma, cutaneous T-cell lymphoma, large-cell, CD30-positive).

Clinical features
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The clinical picture is not characteristic for this lymphoma type. There are solitary, but mostly in majority or also disseminated, cutaneous or subcutaneous plaques, nodules and/or tumors of different sizes. The typical phased process of mycosis fungoides is missing (important distinguishing feature) as well as the eczematoid changes of mycosis fungoides.

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Dermatology

Neurofibroma

Most frequent neurogenic tumour, which occurs solitary or as part of neurofibromatosis .

Histology
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Diffuse or nodular infiltrates of the skin and subcutaneous fatty tissue are characteristic. Moderate or even missing epidermotropy.

Immunohistologically a T-cell pattern is characteristic: pos. for CD3, CD4, CD43, CD45RO. CD8 positive forms have also been described (own entity from the group of cytotoxic T-cell lymphomas). Typical is a relatively large variability of the cell nuclei at relatively constant cell size. The small "cerebriform" lymphocyte typical of Mycosis fungoides is missing. The proliferative activity is significantly increased.

Diagnosis
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Clinical, histological and molecular diagnostics with detection of the T-cell receptor rearrangement (this allows a differentiation between monoclonal and polyclonal populations)

Therapy
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For the small- to medium-cell variants, an identical treatment regimen as for mycosis fungoides must be used. In the case of large-cell CD30-negative pleomorphic T-cell lymphoma, an analogous therapeutic strategy to that used for large-cell CD30-negative T-cell lymphomas must be adopted (see there).

Progression/forecast
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Small to medium-sized pleomorphic T-cell lymphomas have a good prognosis (comparable to mycosis fungoides).

The prognosis of large pleomorphic T-cell lymphomas must be differentiated (CD30-negative forms are regarded as lymphomas with a high degree of malignancy).

Literature
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Beljaads RC et al (1993) Primary cutaneous CD 30-positive large cell lymphoma. Definition of a new type of cutaneous lymphoma with favourable prognosis. An European multicenter study on 47 cases. Cancer 71: 2097-2104
Breccia M (2002) Cutaneous pleomorphic T-cell lymphoma coexisting with myelodysplastic syndrome transforming into acute myeloid leukemia: successful treatment with a fludarabine-containing regimen. Eur J Haematol 68: 1-3
Gonzales CL et al (1991) T-cell-lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemiphagocytic syndrome. On J cathode 15: 17-22
Holloway KB et al (1992) Therapeutic alternatives in cutaneous T-cell lymphoma. J Am Acad Dermatol 27: 367-378
Kim YC (2001) Primary cutaneous pleomorphic small/medium-sized T-cell lymphoma in a young man. Br J Dermatol 144: 903-905
Sterry W et al (1995) Cutaneous malignant lymphomas. Z Hautkr 70: 781-788
by the Driesch P, Coors EA (2002) Localized cutaneous small to medium-sized pleomorphic T-cell lymphoma: a report of 3 cases stable for years. J Am Acad Dermatol 46: 531-535
Willemze R et al (1997) EORTC classification for primary cutaneous lymphomas: a proposal from cutaneous lymphoma study group of the European Organization for Research and Treatment of Cancer. Blood 90: 354-371