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

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 surgeryObstruction/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

Anaplastic T-cells, plasma cells, eosinophils

CD3

TCR gamma - PCR

TGGE

ABI PRISM

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!

VariantsCommonLymphohistiocyticSmall cell