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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 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

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

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9 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

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

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

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16 Sezary’s Syndrome Definition: Micro:
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

17 Peripheral T cell lymphoma
Adult T cell lymphoma Extranodal NK/T cell lymphoma of nasal type

18 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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