Primary cutaneous lymphomas (PCL) are defined as clonal proliferations of malignant T or B lymphocytes that present in the skin with no evidence of extracuta-neous disease at the time of diagnosis. They can be distinguished into cutaneous T-cell lymphomas (CTCL), cutaneous B-cell lymphomas (CBCL) and the rare precursor dendritic cell neoplasms. The PCL are now classified in the new WHO-EORTC classification (Willem-ze et al. 2005; Table 14.1).

Primary cutaneous lymphomas are different from lymphomas of nodal origin in regard to clinical picture, course of disease and therapy. As there has been no curative therapy hitherto, treatment should be directed towards improvement of quality of life. Initial aggressive therapy, i.e. multi-agent chemotherapy, will in most cases result in only a transient remission and is not able to improve overall survival. Therefore an intensive search for new therapeutically active agents, which are acting specifically on the tumour cells, is ongoing. Immune therapies are of special interest as the tumour cells originate from B or T lymphocytes (Table. 14.2). For these cells a whole variety of surface antigens are well defined and monoclonal antibodies are available. In this chapter we briefly describe the

Table 14.2. Biologics in cutaneous lymphoma

Table 14.1. WHO-EORTC classification of cutaneous lymphomas with primary cutaneous manifestations

Cutaneous T-cell and NK-cell lymphomas

Mycosis fungoides

Mycosis fungoides variants and subtypes Folliculotropic MF Pagetoid reticulosis

• Granulomatous slack skin S£zary syndrome

Adult T-cell leukaemia/lymphoma

Primary cutaneous CD30-positive lymphoproliferative disorders

• Primary cutaneous anaplastic large cell lymphoma Lymphomatoid papulosis

Subcutaneous panniculitis-like T-cell lymphoma

Extranodal NK/T-cell lymphoma, nasal type

Primary cutaneous peripheral T-cell lymphoma, unspecified

• Primary cutaneous aggressive epidermotropic CD8-posi-tive T-cell lymphoma (provisional)

• Cutaneous y/8 T-cell lymphoma (provisional)

• Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (provisional)

Cutaneous B-cell lymphomas

Primary cutaneous marginal zone B-cell lymphoma Primary cutaneous follicle centre lymphoma Primary cutaneous diffuse large B-cell lymphoma, leg type Primary cutaneous diffuse large B-cell lymphoma, other intravascular large B-cell lymphoma

Precursor hematologic neoplasm

CD4+/CD56+ hematodermic neoplasm

Molecular structure

DAB389IL-2 (Ontak) Alemtuzumab (MabCampath) 90Y-ibritumomab tiuxetan

(Zevalin) Rituximab (Mabthera) Depsipeptide (FR901228) Suberoylanilide hydroxamic acid

CD25 CD52 CD20


Histone deacetylases Histone deacetylases

Fusion protein CTCL

Monoclonal antibody CTCL

Monoclonal antibody, CBCL

chelator and radionuclide

Monoclonal antibody CBCL

Cyclic tetrapeptide CTCL

Hydroxamic acid CTCL

clinical picture and course of the most common cutaneous lymphomas. We also review data on treatment, efficiency and safety of biological agents currently used in the treatment of PCL.

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