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Extranodal NK/T-Cell Lymphoma, Nasal-Type

  • Amrita Goyal
  • Joi B. Carter
  • Jeffrey A. Barnes
  • Aliyah R. Sohani
  • Lyn McDivitt DuncanEmail author

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.

Keywords

Extranodal NK/T-cell lymphoma, nasal-type NK cell Cytotoxic T cell Nasal Opportunistic infection Hemophagocytic syndrome Disseminated intravascular coagulation Angiocentric Malignant midline reticulosis Midline granuloma Angioinvasive Angiodestructive Differential diagnosis Hydroa vacciniforme-like lymphoma Lymphomatoid granulomatosis CD8+ aggressive epidermotropic cutaneous T-cell lymphoma Blastic plasmacytoid dendritic cell neoplasm 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Amrita Goyal
    • 1
  • Joi B. Carter
    • 2
    • 3
  • Jeffrey A. Barnes
    • 4
  • Aliyah R. Sohani
    • 5
  • Lyn McDivitt Duncan
    • 6
    Email author
  1. 1.Harvard-MIT Division of Health Sciences and TechnologyHarvard Medical SchoolBostonUSA
  2. 2.Department of DermatologyMassachusetts General HospitalBostonUSA
  3. 3.Department of MedicineHarvard Medical SchoolBostonUSA
  4. 4.Comprehensive Cutaneous Lymphoma Program,Department of MedicineMassachusetts General Hospital Cancer CenterBostonUSA
  5. 5.Hematopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  6. 6.Dermatopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA

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