t cell and nk-cell neoplasms
TRANSCRIPT
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T-cell and NK-cell neoplasms
DrAbdullah A.Hashish
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Pre-test.
• What is the role of stem cell differentiation stage in the lymphoid classification? Give examples?
• True or False:– Hodgkin lymphomas are derived from neoplastic T-cell.– B-cell leukemias/lymphomas are more aggressive than T-
cell leukemias/ lymphomas.– NK-cell lymphomas are more aggressive than B-cell
lymphomas.• Short account about Sezary syndrome.
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Post-natal lymphopoiesis in human
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NK cells
• NK cells are defined as:1. cytotoxic cells. 2. with the predominant morphology of large granular
lymphocytes (LGLs). 3. neither productively rearrange any of the genes encoding
the T-cell receptor (TCR) chains 4. nor express on their surface the CD3-TCR complex5. express the CD56 (N-CAM), CD335 (NKp46), and CD16
(FcγRIIIA), antigens in humans.6. can kill cells not expressing major histocompatibility
complex (MHC) class I or class II antigens.
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Morphology of NK cells
• Size of LGLs: medium- to large.• Nucleus: round or indented. • Chromatin: condensed.• Nucleoli: usually prominent.• Cytoplasm: abundant and contains a variety of
organelles and granules.• many NK cells have the morphology typical of LGL, a
significant proportion of NK cells are agranular and indistinguishable from other lymphocytes.6
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T-cell neoplsms
1.Immature.1.Lymphoblastic
leukemia/lymphoma.2.Mature.– Different subtypes.
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T-cell lymphoblastic leukemia/lymphoma
•Morphology:–Size: Medium to large cells.–Chromatin: finely stippled.–Cytoplasm: scanty.
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T-cell lymphoblastic leukemia/lymphoma
• Immunophenotyping:– TdT+, – cytoplasmic CD3+,– CD7+, – CD34+/-, CD2+/-CD1a+/-, CD5+/-,– CD4+/CD8+ or CD4-/CD8-– CD10-/+.
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T-cell lymphoblastic leukemia/lymphoma
• Genotyping:–Abnormalities in:1. TCR loci at:
1. 14q11 (TCR-α), 2. 7q34 (TCR-β), or 3. 7p15 (TCR-γ),
2. t(1;14)(p32-34; q11) involving TAL1
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Mature T and NK cell neoplasms
1. Leukemias: 1. T-cell prolymphocytic leukemia. 2. T-cell large granular lymphocytic leukemia.
2. Lymphomas/Lymphoproliferative Disorders:– Many subtypes.
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T-cell prolymphocytic leukemia
• Morphology:– Size: Small to medium. – Cytoplams: cytoplasmic protrusions or blebs.
• Immunophenotyping:– TdT-, – CD2+, CD3+, CD5+, CD7+, – CD4+ and CD8- is more common than CD4- and
CD8+, but can be CD4+ and CD8+
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T-cell large granular lymphocytic leukemia
• Morphology:– Cytoplasm: • Abundant.• Sparse azurophilic granules
• Immunophenotyping:– CD2+, CD3+, CD5+, CD7+,– CD8+/-, CD16+/-, CD57+/-, – CD4 -/+,– CD56-.
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Lymphomas/Lymphoproliferative Disorders
• Extranodal T/NK-cell lymphoma, nasal type “angiocentric lymphoma”–Immunophenotyping:• CD2+, cytoplasmic CD3+, CD7, CD56,CD4-, CD8-
& EBV+.–Genotyping:• TCR rearrangements usually NEGATIVE• EBV present by in situ hybridization.
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Lymphomas/Lymphoproliferative Disorders
• Cutaneous T-cell lymphoma (mycosis fungoides):– Morphology:• Size: Small to large cells.• cerebriform nuclei.
– Immunophenotyping:• CD2+, CD3+, CD4+, CD5+, CD26+• CD7+/-, CD8-, CD25-.
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Lymphomas/Lymphoproliferative Disorders
• Sezary syndrome:–mostly typical as Cutaneous T-
cell lymphoma (mycosis fungoides)
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Lymphomas/Lymphoproliferative Disorders
• Adult T-cell leukemia/lymphoma:–Morphology: •Highly pleomorphic with multilobed
nuclei.–Immunophenotyping:•CD2+, CD3+, CD5+, CD25+, CD7-, •CD4+CD8- more often than CD4-CD8+
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Lymphomas/Lymphoproliferative Disorders
• Hepatosplenic T-cell lymphoma:–Morphology:• Size: Small to medium cells.• Chromatin: condensed.• round nuclei.
–Immunophenotyping:• CD2+, CD3+, CD5+, CD7+/-• CD4-, CD8+/-.
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Lymphomas/Lymphoproliferative Disorders
• Enteropathy associated T cell lymphoma:–Morphology: • Size: Medium to large cells.• prominent nucleoli.• Cytoplasm: abundant pale, invading mucosal
membranes of the small intestine.– Immunophenotyping: • CD3+, CD7+, CD 103+, CD30+ (most cases),• CD8+/-, CD4-, CD5-, TCRβ+/-.
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NATURAL KILLER (NK) CELL NEOPLASMS
• Large granular lymphocytic leukemia.• Aggressive NK-cell leukemia.• Extranodal NK-cell lymphoma, nasal type “angiocentric
lymphoma”.• Immunodeficiency-associated lymphoproliferative
disorders.– Lymphoproliferative disorders associated with primary
immune disorders. – Lymphomas associated with HIV infection.
• Post-transplant lymphoproliferative disorders (PTLDs).
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NATURAL KILLER (NK) CELL NEOPLASMS
• Large granular lymphocytic leukemia:– Morphology: • Cytoplasm: Abundant cytoplasm and sparse azurophilic
granules.– immunophenotyping: • TdT-, CD2+, CD 7+, CD11b+, CD16+, CD56+,• CD3-, CD4-, CD5-/+, CD8-/+, CD57+/-.
– Genotyping: No TCR rearrangement• Aggressive NK-cell leukemia (show the same but
EBV present).
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NATURAL KILLER (NK) CELL NEOPLASMS
• Extranodal NK-cell lymphoma, nasal type “angiocentric lymphoma”–Immunophenotyping: •CD2+, cytoplasmic CD3ε +, CD 56, CD 7+•CD4-, CD5-/+, CD8-.
–Genotyping:•No TCR rearrangement, EBV present.
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NATURAL KILLER (NK) CELL NEOPLASMS
• Post-transplant lymphoproliferative disorders (PTLDs):–Early lesions: plasmacytic hyperplasia
(PH) and infectious mononucleosis (IM)-like.–Polymorphic PTLD.–Monomorphic PTLD.
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NATURAL KILLER (NK) CELL NEOPLASMS
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NATURAL KILLER (NK) CELL NEOPLASMS
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