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T-cell lymphoma. A. Precursor T-cell lymphomas T-acute lymphoblastic leukaemia T-lymphoblastic lymphoma B. Mature T-and NK cell neoplasms uncommon –10% of all NHL Most frequent : peripheral T-cell lymphoma, unspecified large cell anaplastic lymphoma. Most common T-cell lymphomas.
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T-cell lymphoma A. Precursor T-cell lymphomas • T-acute lymphoblastic leukaemia • T-lymphoblastic lymphoma B. Mature T-and NK cell neoplasms • uncommon –10% of all NHL • Most frequent : peripheral T-cell lymphoma, unspecified • large cell anaplastic lymphoma
Most common T-cell lymphomas 1. Leukaemic/disseminated adult T-cell leukaemia - HTLV 1 2. cutaneous - mycosis fungoides, Sezary syndrome, primary cut. anaplast. lymphoma 3. other extranodal - extranod. NK/T - nasal, enteropathy assoc. 4. nodal - peripheral T-cell lymphoma, NOS, anaplastic large cell lymphoma
Leukaemic • T LGL indolent • ATLL • LA., PB, variants
Mycosis fungoides • Most common cutaneous T-cell lymphoma • middle aged to elderly – males (M:F 2:1) • Protracted • Scaly rash, localized, trunk • Clinical appearancePatches, plaques, tumours, generalized erythroderma • Sezary syndrome: gen. erythroderma, lymphadenopathy, PB involvement
Mycosis fungoides • Indolent • Skin targeted therapy • Chemotherapy for advanced stage
NK/T-cell lymphoma of nasal type • EBV, angiocentric, destructive • South America, Asia Lethal midline granuloma: NK/T-cell l. Wegener granulomatosis Cocaine abuse
coeliac sprue (adults) intestinal perforation intraepitelial T-lymphocytes bad prognosis Enteropathy associated T-cell lymphoma
Enteropathy-associated T-cell lymphoma Proximal jejunum Very rare x most common GI T-cell lymphoma • Acute abdomen (40%) – emergency surgery Obstruction/perforation, peritonitis, sepsis, death • Non-acute: pain, weight loss, malabsorption Age 60, M=F
Enteropathy-associated T-cell lymphoma Multifocal ulcers
Enteropathy-associated T-cell lymphoma Striking association with celiac disease Histology and immunomorphology • Anaplastic/pleomorphic (80%) Celiac dis.+, enteropathy +, CD56- • Monomorphic (20%) Celiac dis.-, enteropathy+/-, CD56+ Half of the patients die soon after the manifestation
Enteropathy-associated T-cell lymphoma CD3 T-cells, plasma cells, eosinophils Anaplastic
TCR gamma - PCR ABI PRISM TGGE Enteropathy assoc. T-cell lymphoma CGH marker: 9q gain (70%; Zettl 2007)
Peripheral T-cell lymphoma, NOS • Lennert lymphoma • Angioimmunoblastic T-cell lymphoma
Anaplastic large T-cell lymphoma • Anaplastic, pleomorphic cells • Hallmark cells, horseshoe-shaped nuclei • CD30, EMA, cytotoxic granule-associated proteins • ALK (ALCL kinase) !SYSTEMIC x CUTANEOUS! Variants Common Lymphohistiocytic Small cell