• By the end of this session you should know:– How to classify T cell lymphoma– The clinical and pathological features of

• Anaplastic large T cell lymphoma

• Mycosis fungoides

Classification of Non-Hodgkin Lymphoma (selected common entities)

• Precursor B cell– Acute lymphoblastic

lymphoma

• Peripheral B cell– Small lymphocytic

lymphoma SLL, Chronic lymphocytic leukemia CLL

– Mantle cell lymphoma– Follicular lymphoma– Marginal zone lymphoma– Diffuse large B cell

lymphom– Burkitt lymphoma

• Precursor T cell– Acute lymphoblastic

lymphoma

• Peripheral T cell– Anaplastic large T cell

lymphoma

– Peripheral T cell lymphoma

– Mycosis fungoides

T cell lymphoma/ NK cell lymphoma

• Rare (less common than B cell lymphoma)

• TCR: alpha,Beta Gamma,delta

• Alpha,Beta: helper and cytotoxic

• Gamma,delta: 1st line of immunity

• NK cells

• Clonality

T cell lymphoma/ NK cell lymphoma

• Generally aggressive tumors

• Clinically can be: nodal, extranodal, cutaneous, leukemic

• More common in Asia

• HTLV1 (Japan, Caribbean)

• Treatment?

Anaplastic large T cell lymphoma

• Clinical: Systemic, Cutaneous– Systemic can be Alk1 pos or Alk1 neg

• Aggressive

• Pathology: mixture of cells, Hallmark cells: large cells with horseshoe nucleus.

• Immuno: CD3, CD30, Alk1 +/-

• Genetic: t(2;5) NPM-Alk1 fusion product

Mycosis Fungoides

• Epidermotropic T cell lymphoma characterized by a proliferation of small or medium-sized neoplastic T lymphocytes with cerebriform nuclei

• Indolent clinical course– Elderly, limited to skin (progress late the course) scaly

eruption

– slow progression over years

– patches, plaques, tumors, lymph node, internal organs

Pathology

• Epidermotropic, band-like infiltrates involving the papillary dermis

• small, medium-sized, and occasionally large mononuclear cells

• hyperchromatic, indented (cerebriform) nuclei

• Pautrier’s microabscesses

• CD3+, CD4+• 5-year survival 87%• Therapy:

– confined to skin: skin-targeted therapies, phototherapy, topical nitrogen mustard, radiotherapy

• Progression to CD30+ or CD30- Large T-cell lymphoma

Sezary’s Syndrome• Definition:

– erythroderma, generalized lymphadenopathy, and the presence of neoplastic T cells in skin, lymph nodes and peripheral blood

• Micro:– may be similar to MF

– more often monotonous cells

– CD3+, CD4+

• Survival: 11%, Chemo is the treatment

• Peripheral T cell lymphoma

• Adult T cell lymphoma

• Extranodal NK/T cell lymphoma of nasal type

• By the end of this session you should know:– How to classify T cell lymphoma– The clinical and pathological features of

• Anaplastic large T cell lymphoma

• Mycosis fungoides