by the end of this session you should know: –how to classify t cell lymphoma –the clinical and...
Post on 21-Dec-2015
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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
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