t- cell lymphomas
TRANSCRIPT
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T- CELL
NEOPLASMS
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Case No 8
8 yrs old female presented with cervical
lymphadenopathy
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TdT
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CD3
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CD20
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CD79a
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Precursor T lymphoblastic
leukemia / lymphomaThis neoplasm is typically composed of small
to medium-sized blast cells with scant
cytoplasm, moderately condensed todispersed chromatin and inconspicuous
nucleoli. Nuclear convolutions may be
prominent and mitotic figures are numerous.
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Epidemiology
Precursor T-ALL (Leukemia) comprises
about 15% of childhood ALL. It is more
common in adolescents than youngerchildren and more common in males than
females.
Precursor T lymphoblastic lymphoma
comprises approx 85 90% of
lymphoblastic lymphomas
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Immunophenotype
Pan T (UCHL1) +
Cytoplasmic CD3+ (linage specific)
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Case No 9
40 years old male presented with
erythematous, scanty and pruritic lesions
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Mycosis Fungoides (MF)
MF is a mature T-cell lymphoma presenting in
the skin with patches / plaques and
characterized by epidermal and dermalinfiltration of small or medium sized T-cells
with cerebriform nuclei.
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So-called Pautrier micro abscesses
Consisting of aggregates of cerebriform cells
in the epidermis are highly characteristic but
are only seen in proportion of the cases.Epidermal involvement with single cell
exocytosis is more common. In the dermis,
infiltration may be patchy, band-like ordiffuse depending upon the stage of the
disease.
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Immunophenotype / Genetics
Pan T (UCHL1) ----- +
CD3 ----- +
CD4 ----- +
CD8 ----- -
T cell receptor genes are clonally
rearranged in most cases.
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Case No 10
40-year-old woman with lymph node
enlargement
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Anaplastic Large Cell Lymphoma
(ALCL)ALCL is a T-cell lymphoma consisting of
lymphoid cells that are usually large with
abundant cytoplasm and pleomorphic oftenhorseshoe shaped nuclei.
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H&E stained ALCL section. Mag: 40X
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Epidemiology
ALCL accounts for approx 3% of adult NHLS
and 1030% of childhood NHLS. ALK-
positive ALCL is most frequent in the firstthree decades of life and shows a male
preponderance
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Immunophenotype / GeneticsCD30 ----- +
ALK ----- +/-
EMA ----- +
UCHL1 / CD3 ----- +/-
Approx 90% of ALCLs show clonalrearrangement of the T-cell receptor genes
irrespective of whether they express T-cell
antigens or not.
Immunoperoxidase stain on a section of ALCL with CD 30 (Ki 1) antibody Note
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Immunoperoxidase stain on a section of ALCL with CD 30 (Ki-1) antibody. Note
Strong positive membrane labeling of large atypical cells. Mag: 20X
Immunoperoxidase stain on a section of ALCL with ALK protein
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Immunoperoxidase stain on a section of ALCL with ALK protein.
Note characteristic nuclear and cytoplasm staining of anaplastic cells. Mag: 40X
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Case No 11
50 years old male presented with
generalized lymphadenopathy and hepato-
splenomegaly.
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Angioimmunoblastic T-cell
Lymphoma (AILT)AILT occurs in the middle aged and elderlywith an equal incidence in males and females.
It is one of the more common specific
subtypes of peripheral T cell lymphomas,
accounting for approx 15
20% of cases or 1
2% of all NHLs.
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Sites of Involvement
Patients usually present with generalized
peripheral lymphadenopathy,
hepatosplenomegaly and frequent skin rash.
BM is commonly involved upon biopsy.
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Clinical Features
AILT usually presents with advanced stage
disease, systemic symptoms and polyclonal
hyper-gammaglobulinemia.
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Histopathology
The lymph node architecture is partially
effaced with regressed follicles. Theparacortex is diffusely infiltrated by apolymorphous population of small to medium
sized lymphocytes usually with clean to palecytoplasm and distinct cell membranes. Theabnormal lymphoid cells are admixed withsmall, reactive lymphocytes, eosinophils,
plasma cells, histiocytes and increasednumber of follicular dendritic cells. Highendothelial venules are abundant and showarborisation.
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Immunophenotype / Genetics
Pan T (UCHL1) +
CD3 +
T cell receptor genes are rearranged in 75%
of the cases.
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Prognosis
The clinical course is aggressive with a
median survival of less than 3 years.
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Case No 12
50 yrs old male presented with cervical
lymph node enlargement
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http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675(06)70453-3&figureId=fig1 -
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Peripheral T cell Lymphoma
(PTCL) unspecifiedA number of distinctive entities have been
defined which correspond to recognizable
subtypes of T-NHL. Once these have beenseparated, there remains a large group of
predominantly nodal (and occasionally extra
nodal) T cell lymphomas which constitute asignificant proportion of the PTCL. We
collectively refer to them as PTCL.
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Epidemiology
These tumors accounts for approx half of
the peripheral T-cell lymphomas. Most
patients are adults but children may also beaffected M:F ratio is 1:1
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Site of involvement
Most patients present with nodal
involvement but any site may be affected
and patients often have generalized diseasewith infiltration in the BM liver, spleen and
extra nodal tissues like skin.
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Histopathology
These lymphomas show diffuse infiltrates.
The cells in most cases are medium sized or
large with irregular, pleomorphic nucleiwhich may be hyperchromatic or vesicular
with prominent nucleoli and many mitosis.
Clear cells and RS like cells are oftenpresent. High endothelial venules are
increased and arborising vessels may be
seen.
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Immunophenotype / Genetics
Pan T (UCHL1) +
CD3 +
CD4 +CD8 -
CD30 +/-
T cell receptor genes are clonally rearranged inmost cases
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Prognosis
They are among the most aggressive of
NHLs. Patients often respond poorly to
therapy, relapses are frequent and OS at 5years is low (20 30%)