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Cutaneous Manifestations Of Systemic Disease Melissa > Cutaneous T cell lymphoma > Flashcards

Cutaneous T cell lymphoma Flashcards

1
Q

Distribution of pagetoid reticulosis MF and treatment

A

distal extremities, is indolent

Tx: radiotherapy or excision

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2
Q

Examination in CTCL

A 
FSE
look at bathing suit distribution for patches/plaques/tumours, hypopigmented/hyperpigmented areas, folliculocentric, atrophy
Look in intetriginous areas. 
Look for poikiloderma
Lymph nodes
HSM
look at Body weight, cachexia, signs of anaemia eg pallor of conjunctiva 
evidence of secondary infection 
alopecia, PPK, onychodystrophy.
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3
Q

poor risk factors for MF with large cell transformation

A 
Generalised skin lesions, early onset of transformation. 
Elevated LDH, beta2 macroglobulin
extracutaneous disease
CD30 neg
folliculotrophic MF
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4
Q

What is woringer kolopp

A

Pagetoid reticulosis

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5
Q

Clinical features of sezary syndrome

A

erythroderma, lymphadenopathy, sezary cells in skin, lymph nodes and peripheral blood

pruritis, alopecia, onychodystrophy, PPK

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6
Q

Aetiology of MF

A

chronic antigen stimulation leading to uncontrollable clonal expansion of T cell helper cells which home to the skin

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7
Q

Leukemia cutis most often seen with

A

myelomonocytic leukemia, T cell malignancies

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8
Q

What is stage IIA MF

A

any patches or plaques with a clinically evident node

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9
Q

T/F in MF TH2 cytokines predominate in early MF

A

F TH1

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10
Q

DDx of jessners

A 
plaque form PMLE
LE tumid
GA, granuloma faciale
cutaneous lymphoid hyperplasia
Cutaneous lymphoma
REM 
fixed drug
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11
Q

20% of subcuneous panniculitis like T cell lymphoma is associated with what and needs to be distinguished from what

A

associated with autoimmune disease

needs to be distinguished from lupus panniculitis

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12
Q

Size differences between small plaque and large plaque parapsoriasis

A

5cm

unless finger like - can by 10cm in long axis

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13
Q

T/F clonality in MF is most likely gamma and beta

A

T

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14
Q

Investigations in MF

A

fbc, film (sezary cells), elfts (esp LDH), T cell subsets (look for CD4:CD8 10:1), flow cytometry, TCR, Biopsy

Consider CT pet/ct
need excision biopsy + BM aspirate

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15
Q

What is stage IIIB MF

A

erythroderma + B1 blood

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16
Q

Granulomatqous MF and granulomatous slack skin is associated with what

A

hodgkins in 30%v

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17
Q

T/F CD 8+ in MF is associated with hyperpigmented lesions

A

F hypopigmented

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18
Q

5 year survival of folliculotrophic MF

A

85%

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19
Q

20% of LyP are associated with

A

MF, CALCL or Hodgkin

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20
Q

B1

A

> 5% of atypical lymphocytes on peripheral smear but

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21
Q

If you have a CD 30+ MF what do you need to distinguish it from

A

1) systemic ALCL
2) CD30+ transformed MF
3) other times of CTCL which sometimes express CD30
4) reactive infiltrates eg viral infections, bites, scabies, AD, HIV or post transplant lymphoma

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22
Q

Tx for LyP

A 
nothing 
MTX, dapsone
UVB
topical steroids
but will likely recur post
could try Radiotherapy 
need for long term FU due to risk of progression - FU 6/12
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23
Q

Tx for C-ALCL

A

refer to exclude systemic

radiotherapy or low dose MTX or excision

24
Q

Types of indolent CTCL

A

MF, CD 30+ lymphoproliferative disease (anaplastic large cell lymphoma & LyP)
Subcutaneous panniculitis like T cell lymphoma, CD4+ small/medium pleomorphic T cell lymphoma.

25
Q

B2

A

a clone which is the same as in the skin and >5% sezary cells or several fold increase in normal CD4:8 ratio, or increase in malignant cells as measured by CD26 or CD7 drop out

26
Q

Stages III and IV

A

TSEBT, interferons, bexarotene, ECP

Vorinostat, romidepsin, denileukion diftitox

27
Q

Bloods of sezary

A

B1>5% sezary cells on buffy coat smear

B2>20% total lymphocyte count or total sezary count of >1000x10^9

28
Q

Dx of large cell transformation of MF

A

cells >4x size of a small lymphocytes and composing > 25%. More likely to occur in higher stages

29
Q

Tx of follicuotrophic MF

A

less responsive to skin directed therapies
PUVA, topical nitrogen mustard
PUVA + IFN alpha or retinoids, local radiotherapy or TSEB

30
Q

Clinical features of folliculotrophic MF

A

more aggressive
AA like, classical type, lichen spinulosis type, infiltration in plques or tumorus.
Acneiform, comedones, nodular prurigo like
hair follicles
pruritic, more severe, often secondarily infected

31
Q

Risk of progression to MF in large plaque parapsoriasis

A

10-35%, 100% if retiform.

32
Q

What is stage IB MF

A

> 10% patches/plaques

33
Q

Poor prognostic factors of MF

A

lymph node involvement

African American females

34
Q

Associations with lymphocytoma cutis

A

borrelia, medications, vaccinations, metal implants, bites, tattoos

35
Q

Clinical features of LyP

A

chronic, recurrent crops of self healing papulonecrotic or papulonodular skin disease
red brown papules and nodules that may develop central haemorrhage, necrosis and crusting

36
Q

Treatments for MF (general measures)

A

For itch:
- emollietnts, topical steroids, soak and smear, wet dressings, opioids for dressing changes, trial an antihistamine.
SSRIs: paroxetine, mirtazeapine, gabapentin, pregabaline, aprepitant
Naloxone,

Reduce infection risik
bleach baths
swab
nasal mupirocin

37
Q

histo of small plaque parapsoriasis

A

focal hyperkeratosis and parakeratosis

aggregates of morphologically normal CD4 TA cells.

38
Q

What is the 5 year survival of tumour stage MF

A

50%

39
Q

How to distinguish ALCL from systemic

A

Cutaneous: CLA+ve, EMA and ALK neg

opposite in systemic

40
Q

What is stage IIB MF

A

tumour stage, may include nodes and blood involvement

41
Q

What type of large plaque psoriasis virtually all progresses to MF

A

retiform parapsoriasis or parakeratosis variegate or parapsoriasis lichenoides

42
Q

Treatment of lymphocytoma cutis

A

should resolve

topical steroids

43
Q

Clinical features of PLEVA

A

appears in crops, polymorphic.
oedematous pink papule that undergoes central vesiculation and haemorrhagic necrosis. Face usually spared. May have malaise/arthralgia. May heal with scars. Lasts 18/12

44
Q

Tx of sezary

A

ECP, IFN alpha, CHOP or CHOP like

PUVA, topical steroids

45
Q

In MF - usually T cell antigen presentation

A

CD4+ CD45RO+

Loss of T cell surface antigens eg CD2, CD5, and/or CD7

46
Q

Where is extranasal NK/T cell lymphoma found and association?

A

asia, central America and south America.

EBV +ve

47
Q

Therapy of stage IA -IIA

A 
Topical steroids
UVB/PUVA
topical nitrogen mustard
bexarotene
local radiation
combinations of the above
acitretin 
low dose MTX 
IFN alpha
48
Q

5 year survival of LyP

A

100%

49
Q

Types of aggressive CTCL

A

sezary
cutaneous peripheral t cell lymphoma (NOS)- (cutaneous aggressive CD8 t cell lymphoma and cutaneous gamma/delta T cell lymphoma)
Cutaneous NK/T cell lymphoma, nasal type

50
Q

Clinical features of PLC

A

firm, lichenoid papule 3-10mm in diameter. Adherent mica like scale which can be scraped off to reveal a shiny brown surface. Unusual to scar
Lasts 18/12

51
Q

Prognosis of MF with large cell transformation if no risk factors, otherwise?

A

65%

otherwise median survival of 24 months, overall survival 33%

52
Q

When to suspect large cell transformation

A

new nodule in pre-existing patch
multiple scattered papules or nodules
solitary nodule in a pre-existing tumour
CHANGE

53
Q

What is APACHE

A

acral pseudolymphomatous angiokeratoma of children - 2-16yrs. Unilateral grouping of small, red to violet angiomatous papules

54
Q

Clinical features of ALCL

A

solitary or localised nodules or tumours that often develop ulceration
multifocal in 20%

55
Q

10 year survival of primary cutaneous ALCL

A

85%

Cutaneous Manifestations Of Systemic Disease Melissa (5 decks)

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