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Extranodal natural killer/T-cell lymphoma, nasal type: a Spanish multicentric retrospective survey

  • Clinical Report
  • Published:
European Journal of Dermatology

Abstract

Background

Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL) is an aggressive lymphoma with a very low incidence in western populations.

Objective

To review the clinicopathological features and outcome of a multicentre series of ENKTL in Spain.

Materials & methods

A multicentre retrospective study was performed based on cases of ENKTL, collected from 1995 to 2004, from 12 dermatology departments included in the Spanish Lymphoma Study Group. The clinical, histopathological, and evolutive features of all these cases were reviewed.

Results

Eighteen patients (three male, 15 female) with median age of 67 years were included in the study. The onset of lesions occurred in the nasal region in 11 patients and on the skin outside this region in the remaining cases. The observed lesions were clinically heterogeneous, corresponding to papules, plaques, and nodules, with or without ulceration. All patients except four received different polychemotherapy regimens, either alone (n = 11) or in combination with radiotherapy (n = 4). After a variable follow-up period (1-36 months), only two patients remained alive. One patient was recently diagnosed (four months ago) with ENKTL in the nasal region and the other presented with skin-limited disease. The median overall survival was 9.5 months.

Conclusions

The results of this retrospective survey confirm that ENKTL is a rare subtype of lymphoma in the Spanish population. All patients showed an aggressive clinical course and poor prognosis, regardless of the initial clinical presentation. Prospective data on larger series of patients treated homogenously are needed to establish the best treatment modality.

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Author information

Authors and Affiliations

  1. Dermatology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain

    Eduardo Rozas-Mñuoz & María Pilar García-Muret

  2. Dermatology Department, Parc de Salut Mar-Hospital del Mar, Barcelona, Spain

    Fernando Gallardo & Ramon Maria Pujol

  3. Dermatology Department, Hospital General de Valencia, Valencia, Spain

    Amparo Pérez-Ferriols

  4. Dermatology Department, Hospital Universitari de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain

    Octavio Servitje

  5. Department of Dermatology, Hospital Clínic, IDIBAPS, Universitat de Barcelona, Barcelona, Spain

    Teresa Estrach

  6. Dermatology Department, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Islas Canarias, Spain

    Jesus Bastida

  7. Dermatology Department, Hospital Universitario de Salamanca, Salamanca, Spain

    Concha Román

  8. Dermatology Department, Hospital de Cabue˜nes, Gijón, Spain

    Lucia Palacio-Aller

  9. Dermatology Department, Hospital Universitari Sant Joan de Reus, Tarragona, Spain

    Inmaculada Gil

  10. Dermatology Department, Hospital Universitari Arnau de Vilanova, Lleida, Spain

    Rosa Maria Martí

  11. Dermatology Department, Hospital de Sant Joan Despí-Moisès Broggi, Sant Joan Despí, Barcelona, Spain

    David Vidal-Sarró

Authors
  1. Eduardo Rozas-Mñuoz
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  2. Fernando Gallardo
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  13. María Pilar García-Muret
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Corresponding author

Correspondence to Eduardo Rozas-Mñuoz.

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Rozas-Mñuoz, E., Gallardo, F., Pujol, R.M. et al. Extranodal natural killer/T-cell lymphoma, nasal type: a Spanish multicentric retrospective survey. Eur J Dermatol 28, 64–70 (2018). https://doi.org/10.1684/ejd.2017.3205

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  • DOI: https://doi.org/10.1684/ejd.2017.3205

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