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Lymphoma cutaneous t-cell lymphoma pleomorphic small-medium- large-cellC84.4
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
DefinitionThis 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 featuresThis section has been translated automatically.
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.
HistologyThis section has been translated automatically.
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.
DiagnosisThis section has been translated automatically.
Clinical, histological and molecular diagnostics with detection of the T-cell receptor rearrangement (this allows a differentiation between monoclonal and polyclonal populations)
TherapyThis section has been translated automatically.
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/forecastThis section has been translated automatically.
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).
LiteratureThis section has been translated automatically.
- 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