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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 Anaplastic large T cell lymphoma Mycosis fungoides
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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
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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
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T cell lymphoma/ NK cell lymphoma
Generally aggressive tumors Clinically can be: nodal, extranodal, cutaneous, leukemic More common in Asia HTLV1 (Japan, Caribbean) Treatment?
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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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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
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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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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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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
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Peripheral T cell lymphoma
Adult T cell lymphoma Extranodal NK/T cell lymphoma of nasal type
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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 Anaplastic large T cell lymphoma Mycosis fungoides
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