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

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. Precursor B cell Acute lymphoblastic lymphoma Peripheral B cell

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

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  1. 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

  2. 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 Classification of Non-Hodgkin Lymphoma (selected common entities)

  3. 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

  4. T cell lymphoma/ NK cell lymphoma • Generally aggressive tumors • Clinically can be: nodal, extranodal, cutaneous, leukemic • More common in Asia • HTLV1 (Japan, Caribbean) • Treatment?

  5. 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

  6. 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

  7. Pathology • Epidermotropic, band-like infiltrates involving the papillary dermis • small, medium-sized, and occasionally large mononuclear cells • hyperchromatic, indented (cerebriform) nuclei • Pautrier’s microabscesses

  8. 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

  9. 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

  10. Peripheral T cell lymphoma • Adult T cell lymphoma • Extranodal NK/T cell lymphoma of nasal type

  11. 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

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