T- CELL NEOPLASMS

Case No 8

8 yrs old female presented with cervical lymphadenopathy

TdT

CD3

CD20

CD79a

Precursor T lymphoblastic leukemia / lymphoma

This neoplasm is typically composed of small to medium-sized blast cells with scant cytoplasm, moderately condensed to dispersed chromatin and inconspicuous nucleoli. Nuclear convolutions may be prominent and mitotic figures are numerous.

Epidemiology
Precursor T-ALL (Leukemia) comprises about 15% of childhood ALL. It is more common in adolescents than younger children and more common in males than females. Precursor T lymphoblastic lymphoma comprises approx 85 90% of lymphoblastic lymphomas

Immunophenotype

Pan T (UCHL1) + Cytoplasmic CD3+ (linage specific)

Case No 9

40 years old male presented with erythematous, scanty and pruritic lesions

Mycosis Fungoides (MF)

MF is a mature T-cell lymphoma presenting in the skin with patches / plaques and characterized by epidermal and dermal infiltration of small or medium sized T-cells with cerebriform nuclei.

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 or diffuse depending upon the stage of the disease.

Immunophenotype / Genetics
Pan

T (UCHL1) ----- + CD3 ----- + CD4 ----- + CD8 ----- T cell receptor genes are clonally rearranged in most cases.

Case No 10

40-year-old woman with lymph node enlargement

Anaplastic Large Cell Lymphoma (ALCL)

ALCL is a T-cell lymphoma consisting of lymphoid cells that are usually large with abundant cytoplasm and pleomorphic often horseshoe shaped nuclei.

H&E stained ALCL section. Mag: 40X

Epidemiology
ALCL accounts for approx 3% of adult NHLS and 1030% of childhood NHLS. ALKpositive ALCL is most frequent in the first three decades of life and shows a male preponderance

Immunophenotype / Genetics
CD30 ALK EMA UCHL1 / CD3 ----- + ----- +/----- + ----- +/-

Approx 90% of ALCLs show clonal rearrangement 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. Strong positive membrane labeling of large atypical cells. Mag: 20X

Note

Immunoperoxidase stain on a section of ALCL with ALK protein. Note characteristic nuclear and cytoplasm staining of anaplastic cells. Mag: 40X

Case No 11

50 years old male presented with generalized lymphadenopathy and hepatosplenomegaly.

Angioimmunoblastic T-cell Lymphoma (AILT)

AILT occurs in the middle aged and elderly with an equal incidence in males and females. It is one of the more common specific subtypes of peripheral T cell lymphomas, accounting for approx 1520% of cases or 1 2% of all NHLs.

Sites of Involvement
Patients usually present with generalized peripheral lymphadenopathy, hepatosplenomegaly and frequent skin rash. BM is commonly involved upon biopsy.

Clinical Features
AILT usually presents with advanced stage disease, systemic symptoms and polyclonal hyper-gammaglobulinemia.

Histopathology
The lymph node architecture is partially effaced with regressed follicles. The paracortex is diffusely infiltrated by a polymorphous population of small to medium sized lymphocytes usually with clean to pale cytoplasm and distinct cell membranes. The abnormal lymphoid cells are admixed with small, reactive lymphocytes, eosinophils, plasma cells, histiocytes and increased number of follicular dendritic cells. High endothelial venules are abundant and show arborisation.

Immunophenotype / Genetics
Pan T (UCHL1) + CD3 + T cell receptor genes are rearranged in 75% of the cases.

Prognosis
The clinical course is aggressive with a median survival of less than 3 years.

Case No 12

50 yrs old male presented with cervical lymph node enlargement

Peripheral T cell Lymphoma (PTCL) unspecified

A number of distinctive entities have been defined which correspond to recognizable subtypes of T-NHL. Once these have been separated, there remains a large group of predominantly nodal (and occasionally extra nodal) T cell lymphomas which constitute a significant proportion of the PTCL. We collectively refer to them as PTCL.

Epidemiology
These tumors accounts for approx half of the peripheral T-cell lymphomas. Most patients are adults but children may also be affected M:F ratio is 1:1

Site of involvement
Most patients present with nodal involvement but any site may be affected and patients often have generalized disease with infiltration in the BM liver, spleen and extra nodal tissues like skin.

Histopathology
These lymphomas show diffuse infiltrates. The cells in most cases are medium sized or large with irregular, pleomorphic nuclei which may be hyperchromatic or vesicular with prominent nucleoli and many mitosis. Clear cells and RS like cells are often present. High endothelial venules are increased and arborising vessels may be seen.

Immunophenotype / Genetics
Pan T (UCHL1) CD3 + CD4 + CD8 CD30 +/+

T cell receptor genes are clonally rearranged in most cases

Prognosis
They are among the most aggressive of NHLs. Patients often respond poorly to therapy, relapses are frequent and OS at 5 years is low (20 30%)