Doc. MUDr. L. Boudová, Ph. D
T-cell lymphomas
Precursor T-cell lymphomas
- T-acute lymphoblastic leukaemia
- T-lymphoblastic lymphoma
Mature T-and NK cell neoplasms
Derived from mature – postthymic T cells
NK cells closely related, some immunophenotypic and functional properties with T cells
uncommon –10% of all NHL
Most frequent T-cell lymphomas: peripheral T-cell lymphoma, unspecified
large cell anaplastic lymphoma
More common in Asians
Causes mostly unknown
Viral influences: EBV association most often with extranodal NK/T-cell lymphomas, NK-cell
leukaemia
HTLV-1 – adult T-cell leukaemia
Clin: very aggressive, bad prognosis
Exceptions: anaplastic large cell lymphoma, mycosis fungoides
Definition of entities – morphological, immunophenotypic, genetic features – imprecise
Clinical features also important for the definiton The most commonly used T-cell markers:
pan T-cell antigens: CD2, CD3, CD5
CD4: helpers – cytokine secreting
CD8: suppressors cytotoxic reactions
Markers of malignancy of T-cell proliferations
1. morphology
2. immunohistochemistry
3. Mol. gen.: assessment of clonality by PCR – TCR
Characteristic translocations
anaplastic large cell lymphoma: ACAL – t(2, 5)
Classification
Leukaemic/disseminated
T-cell prolymphocytic leukaemia
T-cell large granular lymphocyte leukaemia
Aggressive T-cell leukaemia / lymphoma
Adult T-cell leukaemia / lymphoma
Cutaneous
Mycosis fungoides
Sezary syndrome
Primary cutaneous anaplastic lymphoma
Lymphomatoid papulosis
Other extranodal
Extranodal NK/T cell lymphoma, nasal type
Enteropathy-type T-cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma
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Doc. MUDr. L. Boudová, Ph. D
Nodal
Angioimmunoblastic T-cell lymphoma
Peripheral T-cell lymphoma, unspecified
Anaplastic large cell lymphoma
Neoplasms of uncertain lineage and stage of differentiation
Blastic NK cell lymphoma
Leukaemic - disseminated cases
T-cell prolymphocytic leukaemia (not obligatory to learn about)
rare, lymphocytosis – over 100 x 10 9 /l, aggressive
BM, PB, LN, liver, spleen, skin
anaemia, thrombocytopenia
In LN – diffuse - more in the paracortex, no pseudofollicles
T-cell large granular lymphocyte leukaemia (not obligatory to learn about)
Heterogeneous, most cases indolent
Tumour cell: large granular lymphocyte - abundant cytoplasm, coarse cytoplasmic granules –
azurophilic
Main problem: neutropenia
often autoimmune disease
Aggressive NK – cell leukaemia(not obligatory to learn about)
Aggressive, Asian, syoung
often coagulopathy, haemophagocytic syndrome, multiorgan failure
Adult T-cell leukamia/ lymphoma
Tumor cell: highly pleomorphic cells, size medium - large
Caused by the human retrovirus, human T-cell leukaemia virus type 1 .
Endemic (more important) or sporadic
Endemic: in several parts of the world: Japan, in the Caribbean basin, parts of Central Africa
only minority who infected develop the leukamia
Systemic – widely disseminated – BM. PB, LN, other organs
Several clinical variants with different prognosis
Survival: weeks - 1y
Extranodal
Extranodal NK/T cell lymphoma
Angiocentric, vascular destruction, prominent necrosis
Immunophenotype –/NK or T/
Nasal cavity the most common site
Asia, Central and South America
Presentation: epistaxis – large mutilating lesions /former name: lethal midline granuloma
presents localised, dissemination may occur
also outside the nasal cavity
Associated with EBV
Usually aggressive
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Doc. MUDr. L. Boudová, Ph. D
Enteropathy associated T-cell lymphoma
Uncommon, more frequent in areas with high incidence of coeliac dis.(develops in the
intestine affected by coeliac dis.)
Jejunum or ileum
Adults
Abdominal pain, often intestinal perforation
Multiple ulcers Prognosis poor
Hepatosplenic T-cell lymhoma
Rare, young male
Hepatosplenomegaly
Aggressive
Cutaneous lymphoma
Mycosis fungoides (MF) and Sezary syndrome
Most common primary skin lymphoma
Elderly
Remains limited to the skin for ys
At advanced stages: dissemination – LN, liver. PB, spleen, lungs – late event (does not pccur
always)
Grossly: Patches, plaques, - infiltration enlarges, thickens – tumours
trunk
Rarely:generalised dis – erythroderma
Histologically: Epidermal and dermal infiltration by small to medium.sized T-cells with
cerebriform nuclei
Pautrier abscess: collections of tumour cells in the epidermis
Enlarged LN – dermatopathic LA, later tumor cells
Aetiology unknown
Prognosis: most important factor: extent of the disease
Sezary syndrome
Generalised mature T-cell lymphoma
Erythroderma, generalised lymphadenopathy, neoplastic T-ly in the PB
Pruritus, alopecia, nail diseases
Hist. similar to MF
Cerebriform nuclei
variant of MF, but more aggressive
Primary cut. ALCL
ALCL – exists: systemic or primary cutaneous
cutaneous: At the time of diagnosis limited only to the skin, may disseminate afterwards,
diagnosis (primary cut. /systemic) clinically important
Grossly: nodules, solitary or multicentric; ulcerated
Tumor cell: large, large vesicular nucleus – folded =embryo cell, conspicuous nucleolus
Prognosis favourable: 5ys: 90%, often young
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Doc. MUDr. L. Boudová, Ph. D
Lymphomatoid papulomatosis
Chronic, recurrent, benign course, may progress to lymphoma
NODAL
AIBL – angioimmunoblastic lymphadenopathy
LN - Polymorphous infiltrate, prominence of venules with high endothelium
Rel. common. 50 ys
Gen LA, enlarged L+ S, advanced stage, skin rash,
Systemic symptoms, arthritis, ascite
Immune abnormalities – sec. to the neoplastic process
Aggressive – survival less than 3 ys, succumb to infections
Peripheral T-cell lymphoma, NOS
Common, Adults
LN, often generalised
- BM, L, S, extranodal, may becomeleukaemic
- Often B-symptoms
- Causes unknown
morphological subtypes
T-zone
interfollicular pattern of growth
Lennert – lymphoepithelioid cell variant
Small cells and clusters of epithelioid histiocytes in the infiltrate, EO, other
Among the most aggressive of the NHL
Overall survival rates less than 30%
Anaplastic large cell lymphoma - systemic
Tumor cell: large, horseshoe shaped, large vesicular nucleus – folded =embryo cell,
conspicuous nucleolus
Abundant cytoplasm
CD30 (Ki1)+
ALCL kinase protein
Young male (also older)
Sites of involvement: LN, extranodal sites
Present with advanced stage
Often B-symptoms /fever); rel. favourable prognosis
Translocation t(2, 5)
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