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Extranodal NK/T-Cell Lymphoma, Nasal-Type

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Atlas of Cutaneous Lymphomas

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Abstract

Extranodal NK/T-cell lymphoma (eNK/TCL) is an aggressive Epstein-Barr virus (EBV)–associated lymphoma with a very poor prognosis. It commonly presents in the midline face or skin with large ulcerative nodules, may disseminate rapidly to the viscera and lymph nodes, and is complicated by hemophagocytic syndrome and disseminated intravascular coagulation (DIC). Alternative terms in the literature include angiocentric T-cell lymphoma, malignant midline reticulosis, lethal midline granuloma, polymorphic reticulosis, and angiocentric immunoproliferative lesion [1, 2]. One extremely important point to note is that this is not a lymphoma of natural killer T cells (NKT cells), but rather a neoplasm of NK cells or cells with a cytotoxic T-cell phenotype. Hence they are termed NK/T-cell lymphomas [1]. Some differentiate eNK/TCLs that occur in the aerodigestive tract (nasal eNK/TCL) from those that occur elsewhere (nasal-type), but regardless of the primary site, the histology is identical [1]. This chapter discusses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, molecular characteristics, and differential diagnosis of eNK/TCL. The chapter closes with a clinical case featuring a patient with eNK/TCL arising in the nose with cutaneous metastases.

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Author information

Authors and Affiliations

  1. Harvard-MIT Division of Health Sciences and Technology, Harvard Medical School, Boston, MA, USA

    Amrita Goyal M.D.

  2. Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA

    Joi B. Carter M.D.

  3. Department of Medicine, Harvard Medical School, Boston, MA, USA

    Joi B. Carter M.D.

  4. Comprehensive Cutaneous Lymphoma Program,Department of Medicine, Massachusetts General Hospital Cancer Center, Boston, MA, USA

    Jeffrey A. Barnes M.D., Ph.D.

  5. Hematopathology Service, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Aliyah R. Sohani M.D.

  6. Dermatopathology Service, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Lyn McDivitt Duncan M.D.

Authors
  1. Amrita Goyal M.D.
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  2. Joi B. Carter M.D.
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  3. Jeffrey A. Barnes M.D., Ph.D.
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  4. Aliyah R. Sohani M.D.
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  5. Lyn McDivitt Duncan M.D.
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Correspondence to Lyn McDivitt Duncan M.D. .

Editor information

Editors and Affiliations

  1. Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA

    Joi B. Carter

  2. Division of Health Sciences and Technology, Harvard Medical School, Boston, Massachusetts, USA

    Amrita Goyal

  3. Dermatopathology Service, Massachusetts General Hospital, Boston, Massachusetts, USA

    Lyn McDivitt Duncan

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© 2015 Springer International Publishing Switzerland

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Goyal, A., Carter, J.B., Barnes, J.A., Sohani, A.R., Duncan, L.M. (2015). Extranodal NK/T-Cell Lymphoma, Nasal-Type. In: Carter, J., Goyal, A., McDivitt Duncan, L. (eds) Atlas of Cutaneous Lymphomas. Springer, Cham. https://doi.org/10.1007/978-3-319-17217-0_9

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  • DOI: https://doi.org/10.1007/978-3-319-17217-0_9

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-17216-3

  • Online ISBN: 978-3-319-17217-0

  • eBook Packages: MedicineMedicine (R0)

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