t-cell lymphoma a. precursor t-cell lymphomas -t-acute lymphoblastic leukaemia -t-lymphoblastic...
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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
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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
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Leukaemic
• T LGL indolent
• ATLL
• LA., PB, variants
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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
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Mycosis fungoides
• Indolent
• Skin targeted therapy
• Chemotherapy for advanced stage
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NK/T-cell lymphoma of nasal type
• EBV, angiocentric, destructive
• South America, Asia
Lethal midline granuloma:
NK/T-cell l.
Wegener granulomatosis
Cocaine abuse
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Enteropathy associated T-cell lymphoma
• coeliac sprue (adults)
• intestinal perforation
• intraepitelial
T-lymphocytes
• bad prognosis
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Enteropathy-associated T-cell lymphoma
Proximal jejunum Very rare x most common GI T-cell lymphoma
Acute abdomen (40%) – emergency surgeryObstruction/perforation, peritonitis, sepsis, death
Non-acute: pain, weight loss, malabsorption
Age 60, M=F
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Enteropathy-associated T-cell lymphoma
Multifocal ulcers
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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
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Enteropathy-associated T-cell lymphoma
Anaplastic T-cells, plasma cells, eosinophils
CD3
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TCR gamma - PCR
TGGE
ABI PRISM
Enteropathy assoc. T-cell lymphoma
CGH marker: 9q gain (70%; Zettl 2007)
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•Peripheral T-cell lymphoma, NOS
• Lennert lymphoma
• Angioimmunoblastic T-cell lymphoma
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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!
VariantsCommonLymphohistiocyticSmall cell