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 appearance Patches, 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

Enteropathy associated T-cell lymphoma coeliac sprue (adults) intestinal perforation intraepitelial T-lymphocytes bad prognosis

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 TGGE Enteropathy assoc. T-cell lymphoma 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