Cutaneous T-cell lymphoma Flashcards
(88 cards)
1
Q
Epidemiology of mycosis fungoides
A
- account for 50% of all primary cutaneous lymphomas
- Onset typically in 6th/7th decade, but can occur in tounger patient (hypopigmented macules/patches = most common presentation of juvenile MF)
2
Q
Parhtogenesis of mycosis fungoides
A
3
Q
Clinical features of MF
A
- MF initially likes the buttock!
- skin lesion -> diagnosis of Mf +~ 4-6y (median)
- Progression through patch, plaque and tumor (in a subset of patients) stages - protracted course years to decades. Tumor emblee - present with tumors without preceeding patches/plaques
- Patch stage/ irregular erythematous scaly patches occurring in non sun exposed sites/bathing suit distribution may be pruritic, can have variable atrophy/poikiloderma vasculare atrophicans
- Plaque stage: well-demarcates variably shaped violaceous to red-brown scaly plaques
- May be pruritic
- Infiltrates red-brown nodular, polycyclic, horseshoe shape
- Tumor stage: rapidly enlarging nodules with frequent ulceration, arises in a background of patch and plaque lesions (otherwise unlikely to be MF)
- Rare lymphnode and visceral involvement (more likely if skin tumors/generalized plaques/erythroderma)
4
Q
Histology of mycosis fungoides
A
- Patch stage - epidermotropic, atypical lymphocytes (enlarged with cerebriform, hyperchromatic nuclei) predominatly in the epidermis and lined linear up at DEJ with clear halos surrounding the cells. Superficial dermal band -like/lichenoid lymphocytic infiltrate (predominantly reactive lymphocytes)
Clue to epidermotropism (vs exocytosis) = intraepidermal lymphocytes out of proportion to the degree of spongiosis - Plaque stage: more predominant epidermotropism w/more atypical lymphocytes in dense dermal band-like infiltrate
Intra-epidermal clusters of atypical cells. Pautrier micro-abscesses not always seen. Can have eosinophils/plasma cells/blasts can also have interstisial MF - Tumor stage: increased density and depth of dermal infiltrate of atypical lymphocytes with decreased/absent epidermotropism. Can involve entire dermis +_ subcutis, tumor cells increase number and size with cerebriform nuclei
4) LARGE CELL TRANSFORMATION: Large cell transformation defined by >25% large cells (>4x the size of a mature lymphocyte) +~ CD30 expression (often present but not required for the diagnosis). A|w poor prognosis, but CD30+ better prognosis than CD30~
5) IMMUNOPHENOTYPE
typical phenotype - CD3+/CD4+/CD8- mature T-lymphocytes, CD45RO+
6) Variable loss of pan T-cell markers: CD7 loss most common, but least specific, CD5 and CD2 loss less common but more specific
7) hypopigmented MF (variant) - favours darkly pigmented patients, usually CD4-/CD8+ -> cytotoxic -> more interface change (apoptotic KC) and pigment incontinence explains hypopigm
5
Q
Role of T-cell gene rearrangement in Mycosis fungoides
-werk aan antwoord
A
Histological features of MF often ambiguous in early patch stage -> molecular testing for T-cell receptor gene rearrangement may be useful
However (TCR-GR) may be detected in some non-neoplastic inflammatory dermatoses eap eczema -> correlate clinically and hsitologically
6
Q
Treatment of MF
A
- Depend on stage of disease and general condition of the patient. D/t chronic/recurring nature of disease aim is to improve symptoms + limit roxicity
- Skin directed therapies - preferred in early stages of MF
-Topical/IL corticosteroid, topical cytotoxics (nitrogen mustard), phototherapy, radiotherapy. Systemic multi-agent chemo not helpful in early stages
- if skin therapies alone are not effective, can combine with IFN-alpha or systemic retinoids or novel agents like HDACi
2) systemic chemotherapy - advanced stages nodal/visceral ivolvement
3) Radiotherapy in MF
- total skin electron beam irradiation (TSEB)
-36 Gy over 8-10 weeks in factions
-esp helpful for tumor stage
-SE: erythema, scaling, temporary loss of hair, nail, sweatgland function
-electron beam for single tumors
4) Phototherapy in MF
-PUVA, BB-UVB, NB-UVB, UVA1
-ECP (extracorporalphotopheresis) - erythrodermic MF
- Early stages of Mf - can induce remission
- tumor stage MF -> unlikely to alone be enough, can combine with IFN alpha, systemic retinoids or radiotherapy
5) SYSTEMIC THERAPIES
5.1) Interferons - IFN-alpha, administered subcut 3x weekly. SE: flu-like sympt, hair loss, depression, nausea, BM supression. PUVA + IFN alpha higher response than PUVA alone
5.2) Retinoids : isotret, acitretin, novel bexarotene. If patients have thin patches/plaques topical retinoids may be considered
5.3) Denileukin diftitox - fusion protein where diptheria toxin is linked to IL-2, decrease protein synthesis + cell death. SE: capillary leak syndrome, fever, fluid retention
5.4) Histone de acetylase inhibitors (HDACi)
-inhibit enzyme HDAC-> affect expresiion of many genes, involved in cellular prolif, differentiation, migration, apoptosis. SE= GI symp, theombocytopenia
5.4) Systemic chemotherapy
only if Ln or visceral involvement. 6x cycles of CHOP. Allogeneic hemapoeitic stem cell transplantation in young patients.
7
Q
prognosis of Mf
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8
Q
Write short notes on pagetoid reticulosis
A
9
Q
Write short notes on Granulomatous Slack skin
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10
Q
Write key facts on folliculotropic Mf
A
11
Q
Write short notes on sezary syndrome
-epidemiology
-clinical features
-pathology
A
12
Q
Write short notes on primary cutaneous CD30+ lymphoprolif disease
A
13
Q
immunophenotyping of primary cut anaplastic large cell lymphoma
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14
Q
Genetic features of primary cutaneous anaplastic large cell lynphoma
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15
Q
treatment of primary cutaneous anaplastic large cell lymphoma
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16
Q
Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma
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17
Q
Primary cutaneous peripheral T-cell lymphoma, NOS
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18
Q
Algorithm for the diagnosis and treatment of cutaneous CD30+ lymphoproliferations
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19
Q
picture of primary cut anaplastic large cell lymphoma
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20
Q
Lymphomatoid papulosis (LyP) - histologic subtypes and their differential diagnoses
A
21
Q
Comparison of subcutaneous panniculitis-like T-cell lymphoma and primary cutaneous Gamma/delta T-cell lymphoma with subcutaneous involvement
A
22
Q
Write short notes on 1) thepathogenesis of sezary syndrome
2) diff
3) treatment
A
23
Q
Picture of lymphomatoid papulosis
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24
Q
picture of subcut panniculitis like T-cell lymphoma (SPTLC)
A
25
Picture of nasal NK/T-cell lymphoma
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picture if primary cut CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma
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Picture of primary cut peripheral T-cell lymphoma, NOS
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Picture of Mf
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picture of alopecia mucinosa
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Clinical variants of mycosis fungoides
31
write notes on adult T-cell leukemia/lymphoma
1) subtype: slowly progressive = smoldering. Smoldering -> patches, plaques, papular skin lesions
2) a/w HTLV-1 Virus -> endemic in areas with high virus prevalence (Japan, Carribean, Central Africa)
3) present with leukemia, lymphadenopathy, organomegaly, hypercalcemia, and skin lesions, poor prognosis
4) Histopathology: resembles MF, but has characteristic ‘floret’ or ‘clover-leaf’ malignant T-cells. Small, medium, large pleomorphic T-cells, marked epidermotropism, CD3+, CD4+, CD8-, CD25+
5) Rx: zidovudine+IFN alpha, systemic chemo needed for most cases or anti-CCR4
32
Write short notes on Lymphomatoid papulosis (Lyp)
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PLEVA vs LyP
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Primary cutaneous ALCL (anaplastic large cell lymphoma)
1) Solitary (>multiple) ulcerated tumor/nodules up to 10cm (larger than LyP), usually adults, unlikely LyP, lesions do not rapidly ‘come and go’
2) frequently persists/relapses in skjn, rare nodal involvement
3) Composed of large cells with anaplastic pleomorphic or immunoblastic cytomorhology AND expression of CD30 antigen by >75% of tumor cells. Distinguish from Mf with blastic transformation
4) HISTO: Morphology of anaplastic cells with round/oval irregular nuclei, eosinophilic nucleoli. Sheets of large atypical CD30+ lymphocytes compromising >75% of infiltrate; majority are CD4+
5) Lack ALK translocation (vs systemic ALCL), EMA negative as well
6) Ulcerative lesions - Lyp Like. Histo- inflammation, reactive cells, histiocytes, eos, neutros
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Subcutaneous panniculitis like T-cell lymphoma
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Extranodal NK/T-cell lymphoma nasal type
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Aggressive epidermotropic cytotoxic (CD8+) T-cell lymphoma
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Primary cutanoeus CD4-positive small/medium pleomorphic T-cell lymphoma
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Primary cutaneous B-cell neoplasms
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Notes on leukemia cutis
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Diagnosis of CTCL
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Classification CTCL
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Practical guidelines if CTCL
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Staging of CTCL
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Staging of mycosis fungoides
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Primary cutaneous gamma/delta T-cell lymphoma
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WHO-EORT. classification for cutaneous T-cell lymphomas
50
Algorithm for the classification of cutaneous T-cell lymphoma
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Diffential diagnosis of common histologic patterns in cutaneous T-cell lymphomas (1)
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Differential diagnosis of common histologic patterns in cutaneous T-cell lymphoma (2)
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TNMB classification of mycosis fungoides and sezary syndrome
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Clinical staging system for mycosis fungoides and sezary syndrome
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Treatment of mycosis fungoides
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Picture of MF
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Picture of MF
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picture of MF
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picture if folliculotropic MF
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Picture of pagetoid reticulosis
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Picture of granulomatous slack skin
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Picture of sezary syndrome
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Write short notes on the classification of Lymphoma
64
Write short notes on non-hodgkins lymphoma (tabulate)
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Mycosis fungoides
-defintion
-summary
66
Write short notes on Mf
-epidemiology
-etiology/pathogensis
-clinical presentations
-clinical variants
67
Summary of sezary syndrome (also a variant of MF)
68
Histology of Mf
69
Staging of Mf
70
Diagnosis of Mf
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Summary of Mf treatment (1)
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Summary if MF treatment (2)
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Summary of other CTCL (apart from MF)
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Summary of primary cutaneous anaplastic large cell lymphoma
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Summary of lymphomatoid papulosis (1)
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Summary if Lymphomatoid papulosis (2)
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Subcutaneous panniculitis-like
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Primary cutaneous CD4+ small/medium pleomorphic TCL
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Summary adult T-ce
leukemia
i
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Adult T-cell leukemia/lymphoma
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Primary cutaneous aggressive epidermotropic CD8+ TCL
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Cutaneous gamma/delta TCL
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Summary of extranodal NK/TCL (nasal type) (1)
84
Summary of extranodal NK/TCL
85
Summary if Cutaneous B-lymphomas
86
Summary of Cutaneous B-cell lymphoma (1)
87
Summary of primary cut B-cell lymphoma (2)
88
Types of adult T cell leukemia/lymphoma
