Lymphoma cutaneous nk/t cell lymphoma (overview) C84.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

cutaneous NK/T-cell lymphoma; Extranodal NK/T-cell lymphoma nasal type; Extranodal NK/T cell lymphoma of the nasal type; Granuloma gangraenescens nasi (s.dort); lethal midline granuloma; Lymphoma cutaneous NK/T-cell lymphoma; NK/T-cell lymphoma; NK/T-cell lymphoma of the skin; polymorphic granulomatosis

Definition
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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.

Classification
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  • NK/T-cell lymphoma, nasal type
  • NK/T-cell lymphoma, extranasal type
  • Hydroa-vacciniforme-like T-cell lymphoma

Occurrence/Epidemiology
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More common in Asia, South America, North America. In Europe rarely but regularly found.

Etiopathogenesis
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Associations with infections with the Epstein-Barr virus are frequent.

Manifestation
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  • Nasal infestation: Predominantly in younger adults; men are more frequently affected than women.
  • In case of extranasal infestation: Mean age of the disease is about 50 years; men are affected in a ratio of 3:2 compared to women. Obviously no ethnic preference.

Localization
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  • Nasal NK/T cell lymphomas (older name Granuloma gangraenescens nasi or lethal midline granuloma of the face): Preferably in nose and sinuses.
  • Extranasal NK/T-cell lymphomas: Preferably skin, gastrointestinal tract, testis.

Clinical features
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  • Nasal NK/T-cell lymphomas: They are often accompanied by tumorous masses and centrofacial destruction. Clinical onset with uncharacteristic, purulent, hemorrhagic rhinitis or infections of the upper airways, oedema of the nose, necrosis of the nasal wings and nasal septum, destruction of the paranasal sinuses, ethmoid bone and skull base. Arrosion bleeding, septicopyemic complications, fever attacks. Later dissemination, the lymphoma then manifests itself extranodally at various extranasal organ systems. Secondary infestation of lymph nodes is rare.
  • Extranasal NK/T-cell lymphomas (preferably skin): lymphoma with a relatively poor prognosis; mean survival time: 15 months; solitary or generalized skin infestation; preferably trunk or extremities, rarely face; solitary skin infestation in about 60% of the patients; in about 40% of the patients lymph nodes, the gastrointestinal tract and bones must be expected.
  • A variant that occurs in Asia and Central/South America in children and adolescents is hydroa-vacciniforme-like T-cell lymphoma, which is characterized by vesiculo-bullous lesions and facial edema in light-exposed areas.

Histology
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  • 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 cell nuclei with granular chromatin. In nasal NK/T-cell lymphomas a dense infiltration of reactive cells (lymphocytes, plasma cells, macrophages, eosinophil granulocytes) is often found. Caution! NK/T-cell lymphomas can be misinterpreted as an inflammatory disease; see Granuloma gangraenescens nasi (older name) below.
  • Immunophenotype: CD56 pos. (identical with the neuronal adhesion molecule N-CAM); CD4 pos.; CD123 pos.; CD8 neg.(see also CD-classification)
  • In extranasal infestation pattern with almost no epidermotropy (in about 20% of cases only slight epidermotropism) the following immunophenotypes were found: CD56 pos. (100%); CD20 neg. (100%); CD3 pos. (60%); CD30 pos. (20%); detection of EBV-RNA pos. (30%); MDR1-encoded protein pos. (80%). With regard to the prognosis, only the expression of CD30 seems to be relevant: when CD30 pos. the mean survival time is > 35 months, in CD30-negative forms the mean survival time is about 9.6 months).

Diagnosis
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Clinic, laboratory, histology, EBV detection.

Complication(s)
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Septic infections.

Therapy
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Progression/forecast
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Aggression course with mean survival time of < 3 years.

Literature
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  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
  4. Kakihara T et al (2002) Centrofacial malignant T-cell lymphoma exhibiting recurrent Fever and skin ulcer in a 3-year-old girl. Pediatric Hematol Oncol 19: 575-580
  5. Mraz-Gernhard S et al (2001) Natural Killer/Natural Killer-like T-Cell Lymphoma, CD 56+, presenting in the skin: An increasing recognized entity with an agressive course. J Clin Oncol 19: 2179-2188
  6. Petrella T (2004) A CD56-negative case of blastic natural killer-cell lymphoma (agranular CD4+/CD56+ haematodermic neoplasm). Br J Dermatol 150: 174-175
  7. Russell-Jones R (2003) World Health Organization classification of hematopoietic and lymphoid tissues: implications for dermatology. J Am Acad Dermatol 48: 93-102
  8. Santucci M et al (2003) Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer 97: 610-627

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Last updated on: 29.10.2020