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An extranodal NK/T cell lymphoma, nasal type, with specific immunophenotypic and genotypic features

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Abstract

Extranodal NK/T cell lymphoma, ‘nasal type,’ is a rare clinicopathological entity in Europe. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. We report the case of a patient who presented with fever, dyspnea, nasal congestion, headache, distention of right nasal turbinates and exophytic lower leg ulcerating lesions. A CT scan of visceral scull demonstrated a filling mass of right frontal, ethmoidal and maxillary sinuses with erosion of the wall of right maxillary sinus and ventral portion of the diaphragm. A biopsy was performed in the skin lesion and showed an angioinvasive NK/T cell lymphoma CD56 negative with clonal rearrangement of the T-cell-receptor γ gene. Up to our knowledge, this is a rare immunophenotype for NK/T-cell, ‘nasal type,’ lymphomas. However, the lymphoma may be classified as extranodal NK/T cell lymphoma, ‘nasal type,’ due to typical clinical presentation, radiologic findings and pathological characteristics of polymorphism, angioinvasion, angiodestruction and coagulative necrosis.

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Authors and Affiliations

  1. 2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece

    Panagiotis Katsaounis, Alexandra Alexopoulou, Spyros P. Dourakis, Alexandros Smyrnidis, Anna Filiotou & Athanasios J. Archimandritis

  2. 40 Konstantinoupoleos St., 16342, Hilioupolis, Athens, Greece

    Alexandra Alexopoulou

  3. Pathology of Blood Department, Evangelismos Hospital, Athens, Greece

    Leonidas Marinos

Authors
  1. Panagiotis Katsaounis
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  2. Alexandra Alexopoulou
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  3. Spyros P. Dourakis
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  4. Alexandros Smyrnidis
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  5. Leonidas Marinos
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  6. Anna Filiotou
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  7. Athanasios J. Archimandritis
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Correspondence to Alexandra Alexopoulou.

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Katsaounis, P., Alexopoulou, A., Dourakis, S.P. et al. An extranodal NK/T cell lymphoma, nasal type, with specific immunophenotypic and genotypic features. Int J Hematol 88, 202–205 (2008). https://doi.org/10.1007/s12185-008-0137-9

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  • DOI: https://doi.org/10.1007/s12185-008-0137-9

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