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Extranodal NK/T-cell lymphoma

Extranodal NK/T-cell lymphoma. Monirath Hav, MD, PhD fellow Pathology Department Ghent University Hospital. General overview on hematopoiesis. Relative frequencies of B-cell lymphoma subtypes. Anon. (1997). Blood 89 : 3909-3918. Relative frequencies of mature T-cell lymphoma subtypes.

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Extranodal NK/T-cell lymphoma

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  1. Extranodal NK/T-cell lymphoma Monirath Hav, MD, PhD fellow Pathology Department Ghent University Hospital

  2. General overview on hematopoiesis

  3. Relative frequencies of B-cell lymphoma subtypes Anon. (1997). Blood 89 : 3909-3918

  4. Relative frequencies of mature T-cell lymphoma subtypes Armitage J et al.J Clin Oncol 26 : 4124-4130

  5. Introduction Extranodal NK/T-cell lymphoma • More prevalent in Asians • Often in adults (6th decade) • More common in males (3:1) • > 95% associated with EBV • Sites of involvement: • upper aerodigestive tract (nasal cavity ++++) • Extranasal including skin, soft tissue, GI tract, and testis • Prognosis: aggressive lymphoma • Poor response to chemotherapy (MDR-1 +) • Median survival 12.5 months • High local recurrence rate (21.4% Vs 5% in T cell lymphoma of nasopharynx)

  6. Morphology • Angiocentric & angiodestructive diffuse growth pattern +++ • Fibrinoid changes in Vx +++ • Coagulative necrosis +++ • Admixed apoptotic bodies +++ • Mitotic figures easily found • Most often medium-sized cells • Pale to clear cytoplasm • Irregular, folded nuclei • Inconspicous nucleoli

  7. Immunophenotype • CD2 + • Surface CD3 – but cytoplasmic CD3 + • CD56 + • Granzyme B, TIA1, or perforin + • Occasional cases show CD7 & CD30 + • EBV +

  8. Differential Diagnosis CD56 + lymphoid neoplasms: • Aggressive NK cell leukemia: systemic involvement (PB, LN, BM ++) • Enteropathy-associated T-cell lymphoma: associated with IEL • Hepatosplenic T-cell lymphoma: typical sites of involvement • PCTCL subsets

  9. Case report • 41 M • Obstructing tumour of the nasal cavity • Destruction of septal trabeculae

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