Cytotoxic Lymphomas

Werner Kempf, Dmitry V. Kazakov, Reinhard Dummer, and G√ľnter Burg

Department of Dermatology, University Hospital, Zurich, Switzerland

The spectrum of cytotoxic lymphomas of the skin is heterogeneous and includes CD8+ T-cell, T/NK-cell, and NK-cell lymphomas as well as neoplasms related to myelomonocytic leukemia. As a common hallmark, the tumor cells display cytotoxic proteins. There is no widely accepted classification (and or even nomenclature) of this group of lymphomas. Immunophenotyping plays a crucial role in diagnosing cytotoxic lymphomas. Future developments will clarify, which forms of cytotoxic lymphoma in fact represent cytotoxic variants of well-established CL such as MF and which ones are distinct nosologic entities. In accordance with the basic principle

Table 1 Cytotoxic Lymphomas with Primary or Secondary Cutaneous Involvement

Primary cutaneous CD8+ cytotoxic lymphoma Epidermotropic forms

MF-like hypo- or hyperpigmented types Aggressive type Sezary syndrome-like variant Nonepidermotropic forms

Nodular type Extranodal NK/ T-cell lymphoma Nasal type

Subcutaneous panniculitis-like lymphoma Gamma/delta lymphoma Lymphoblastoid NK-cell lymphoma/leukemia CD4+ CD56+ blastic lymphoma/leukemia Aggressive T=NK cell lymphoma=leukemia

Note: CD30+ lymphoproliferative disorders of the skin such as lymphomatoid papulosis and CD30+ ALCL have usually a CD4+ T-helper cell phenotype, but often express cytotoxic molecules such as TIA-1.

of recent lymphoma classifications (WHO, REAL, and EORTC) to delineate nosologic entities, the aim of this chapter is to describe those forms of cytotoxic lymphomas with primary or secondary skin involvement which display distinct clinical, pathologic, and immunophenotypic features (Table 1). It is essential to identify lymphomas with a cytotoxic phenotype, since some forms exhibit an aggressive course which has therapeutic implications (1-4).

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