1. 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

2. 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

3. Leukaemic
T LGL indolent
ATLL
LA., PB, variants

4. 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

5. Mycosis fungoides Indolent Skin targeted therapy
Chemotherapy for advanced stage

6. NK/T-cell lymphoma of nasal type
EBV, angiocentric, destructive
South America, Asia
Lethal midline granuloma:
NK/T-cell l.
Wegener granulomatosis
Cocaine abuse

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

8. 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

9. Enteropathy-associated T-cell lymphoma
Multifocal ulcers

10. 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

11. Enteropathy-associated T-cell lymphoma
CD3
T-cells, plasma cells,
eosinophils
Anaplastic

12. TCR gamma - PCR TGGE Enteropathy assoc. T-cell lymphoma
ABI PRISM
TGGE
Enteropathy assoc. T-cell lymphoma
CGH marker: 9q gain (70%; Zettl 2007)

13. Peripheral T-cell lymphoma, NOS
Lennert lymphoma
Angioimmunoblastic T-cell lymphoma

14. 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