Primary cutaneous (anaplastic) large cell lymphoma cd30-negative C84.5

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%!

1. 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
2. Dereure O et al (2003) Rare occurrence of CD30+ circulating cells in patients with cutaneous CD30+ anaplastic large cell lymphoma: a study of nine patients. Br J Dermatol 148: 246-251

3. Dueck D (2002) CD8-positive, CD30-negative cutaneous T-cell lymphoma simulating pyoderma gangrenosum. dermatologist 53: 114-117

4. Fauconneau A et al (2015) Assessment of diagnostic criteria between primary cutaneous anaplastic large cell lymphoma and CD30-rich transformed mycosis fungoides. A study of 66 cases. Br J Dermatol doi: 10.1111/bjd.13690
5. Foss HD et al (2000) The many faces of anaplastic large cell lymphoma. Pathologist 21: 124-136
6. Gonzales CL et al (1991) T-cell-lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemphagocytic syndrome. Am J Pathol 15: 17-22
7. Holloway KB et al (1992) Therapeutic alternatives in cutaneous T-cell lymphoma. J Am Acad Dermatol 27: 367-378
8. Kadin ME et al (1994) Primary Ki-1- positive anaplastic large cell lymphoma: A distinct clinicopathological entity. Ann Oncol 5 Suppl 1: 25-30
9. Kang SK et al (2002) Coexistence of CD30-positive anaplastic large cell lymphoma and mycosis fungoides. Clin Exp Dermatol 27: 212-215
10. Mao X et al Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma. Genes Chromosomes Cancer 37: 176-185
11. Mira JA et al (2003) Bone involvement and abcess formation by neutrophil-rich CD30+ anaplastic large-cell lymphoma mimicking skeletal infection in an AIDS patient. J Infect 47: 73-76

12. Nevet MJ et al (2015) Transformation of Sézary syndromeinto
    CD30+ anaplastic large T-cell lymphoma after alemtuzumab therapy withevidence
    of clonal unity. On J Dermatopathy 37:73-77