Primary cutaneous (anaplastic) large cell lymphoma cd30-negativeC84.5

Large cell cutaneous T-cell lymphoma with characteristic nuclear polymorphism and lack of expression of CD30 (activation marker from the group of nerve growth factor receptors). Controversial entity that is considered differently in relevant classifications. In the WHO-EORTC classification, this specificity is listed under "primary cutaneous peripheral T-cell lymphomas, not further specified (NOS)" (see under cutaneous T-cell lymphomas - overview).

This lymphoma species is estimated to account for approximately 7% of all cutaneous T-cell lymphomas.

Occurring in older adults (average age 60 years) (men are slightly more frequently affected:1.5/1.0)

Ubiquitous occurrence possible; especially trunk, extremities, neck, neck.

Typical are nodular or diffuse non-epidermotropic, large-cell, anaplastic infiltrates that cover the entire dermis and partially extend into the subcutis. Infiltrate cells with a large, bright cytoplasm with several nuclear corpuscles and a distinct eosinophilic cytoplasm. High mitotic activity. Associated reactive inflammatory cells (eosinophilic granulocytes, histiocytes, small lymphocytes) are found in the periphery of the tumor infiltrates.

Immunohistologically, the expression of CD30 is missing. T-cell pattern variably absent. The clonal T cell receptor gene rearrangement is positive in the majority of cases. Reactivity of CD15 and EMA (epithelial membrane antigen) is mostly absent.

S.u. Cutaneous T-cell lymphoma.

The prognosis of CD30-negative primary cutaneous anaplastic large T-cell lymphomas is significantly worse than that of CD30-positive primary cutaneous anaplastic large T-cell lymphomas. Thus, the average 5-year survival rate for the former is about 15%.

In contrast, the much more frequent primary cutaneous CD30-positive anaplastic large T-cell lymphoma has a 5-year survival rate of about 90%!

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