Lymphoma cutaneous nk/t cell lymphoma C84.4

Rare, highly malignant lymphoma that is predominantly derived from natural killer cells (NK cells). The skin infestation usually proves to be a secondary infestation of an otherwise systemically spreading lymphoma. Some NK/T-cell lymphomas of the nasal type show an association with the Epstein-Barr virus.

Recently, an origin of immature dendritic cells (veiled cells [IDC cells]) has been postulated due to a frequently detectable CD123 expression of the tumor cells. Since some of these lymphomas are indicative of T-cell descent, they are referred to as NK/T-cell tumors in the REAL and WHO classification.

NK/T-cell lymphoma, nasal type

NK/T-cell lymphoma, extranasal type

Hydroa-vacciniform type T-cell lymphoma

Nasal infestation: Predominantly in younger adults; men are more frequently affected than women.

Extranasal involvement: Mean age of onset about 50 years; males are affected in a ratio of 3:2 compared to females. Apparently no ethnic predilection.

Nasal NK/T-cell lymphoma (older name granuloma gangraenescens nasi or lethal midline granuloma of the face): Prevalent in the nose and sinuses.

Extranasal NK/T-cell lymphoma: Prefers skin, gastrointestinal tract, testis.

Nasal NK/T-cell lymphomas: They are often associated with tumorous space-occupying lesions as well as centrofacial destruction. Clinical onset with uncharacteristic purulent hemorrhagic rhinitis or upper respiratory tract infections, edema of the nose, necrosis formations of the nostrils and nasal septum, destruction of the paranasal sinuses, ethmoid bone, and skull base. Arrosion hemorrhages, septicopyemic complications, febrile episodes. Later dissemination, the lymphoma then manifests extranodally in various extranasal organ systems. Secondary involvement of lymph nodes, however, is rare.

Extranasal NK/T-cell lymphoma (preferentially skin): Lymphoma with a relatively poor prognosis; median survival: 15 months; solitary or generalized skin involvement; preferentially trunk or extremities, rarely face; solitary skin involvement in about 60% of patients; in about 40%, involvement of lymph nodes, gastrointestinal tract and bones must be expected.

A variant occurring in children and adolescents in Asia and Central/South America is hydroa-vacciniform-like T-cell lymphoma characterized by vesiculo-bullous lesions and facial edema at light-exposed sites.

Diffuse spread of lymphoma infiltrate, often with angiocentric and angiodestructive growth pattern and extensive coagulation necrosis. Broad spectrum of tumor cells of various sizes or cell anaplasia. Often irregularly shaped nuclei with granular chromatin. In nasal NK/T-cell lymphomas, a dense infiltrate of reactive cells (lymphocytes, plasma cells, macrophages, eosinophilic granulocytes) is often found. Caveat. NK/T-cell lymphoma can be misinterpreted as an inflammatory disease; see also granuloma gangraenescens nasi (older name).

Immunophenotype: CD56 pos. (identical to the neuronal adhesion molecule N-CAM); CD4 pos.; CD123 pos.; CD8 neg.(see also CD classification)

In the case of extranasal infestation, the following immunophenotype was found with almost no epidermotropy (in about 20% of cases only slight epidermotropism): CD56 pos. (100%); CD20 neg. (100%); CD3 pos. (60%); CD30 pos. (20%); detection of EBV-RNA pos. (30%); MDR1-encoded protein pos. (80%). Regarding prognosis, only CD30 expression seems to have relevance: when CD30 pos. the median survival time is > 35 months, for CD30-negative forms the median survival time is about 9.6 months).

For patients with localized stages, radiotherapy is recommended.

Because of the high rate of systemic recurrences, additional chemotherapy with anthracycline-containing polychemotherapy is indicated.

See also lymphoma, cutaneous T-cell lymphoma.

Aggression course with mean survival time of < 3 years.

1. Acosta AM et al (2016) Post-transplant extranodal NK/T-cell lymphoma, nasal type with cutaneous and pulmonary involvement. Pathology 48:380-383.
2. Halm S et al (2008) agranular CD4/CD8+ hematodermal neoplasia (CD56 positive non-Hodgkin's lymphoma). Act Dermatol 34: 192-195
3. Ichimura K (2003) Phenotypic analysis of peripheral T/NK cell lymphoma: study of 408 Japanese cases with special reference to their anatomical sites. Catholic Int 53: 333-344
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