T-cell lymphoma

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 (MF 21)
• Protracted
• Scaly rash, localized, trunk
• Clinical appearancePatches, 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, MF

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
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