Suárez Porteria Extranodal T/NK lymphoma, nasal type
Transcription
Suárez Porteria Extranodal T/NK lymphoma, nasal type
Club Linfomas Santander Abril 2013 F Climent, V Marco, J González, E González-Barca, O Servitje Hospital Universitari de Bellvitge Clinical history • • • • Woman 63 years old History of chronic sinusitis Cutaneous lesion on the right arm Biopsy BF1 CD3 CD5 CD2 CD4 CD8 CD7 CD56 TIA-1 GR-B KI67 EBERs Extranodal NK/T-cell lymphoma, nasal type CD3 CD2 CD5 CD7 CD4 CD8 Ki67 CD4 CD56 CD8 EBERs 2010 CD20 CD3 CD2 CD7 CD5 CD56 CD4 Ki67 CD8 EBERs Skin 2013 Nasal polyp 2010 Diagnosis • Extranodal NK/T-cell lymphoma, nasal type • Stage IIIA • IPI 3 (Age, stage, extranodal location) • CHOP/14 x 6 Comments • The prognosis of extranodal NK/T-cell lymphoma, nasal type is variable. • Historically, the survival rate has been poor (3040%) • Unfavourable prognostic factors: – – – – – Advanced stage Unfavourable IPI Invasion of bone or skin High circulating EBV DNA Presence of EBV+cells in BM • ENKTL in the United States shares many features with cases from Asia and Central and South America • Prognosis value – High IPI – Extranodal sites of disease – High proliferation rate (Ki 67 >60%) • 140 cases • 6 selected cases had a minimum of 3 relapses and a median overall survival of 66 months • Lower expression of Ki67 (<40-50%) My case • ENKTL of T-cell lineage • Skin lesion Agressive lymphoma • 2010 nasal biopsy?? – Small lymphocytes – T-cell phenotype, CD56 negative – Ki67 very low – Same T-cell clon – Indolent or early lesion??