CUTANEOUS T-CELL LYMPHOMA

Question 1

CUTANEOUS T-CELL LYMPHOMA kinds

Answer 1

Mycosis fungoides ((limited supercial type)
Sézary syndrome (widespread systemic type)

Question 2

Mycosis fungoides Lesion

Answer 2

characterized by erythematous patches/plaques/nodules/tumours, which may be pruritic and
poikilodermic (atrophy, telangiectasia, hyperpigmentation, hypopigmentation)

Question 3

Mycosis fungoides

common sites

Answer 3

trunk, buttocks, proximal limbs

Question 4

Mycosis fungoides how severe it is? and prognosis

Answer 4

mildly symptomatic, usually excellent prognosis for early disease

Question 5

Sézary syndrome Clinical Feature

Answer 5

rare variant characterized by erythroderma, lymphadenopathy, associated with intense pruritus, alopecia, palmoplantar hyperkeratosis, and systemic symptoms
(fatigue, fever)

Question 6

Sézary syndrome blood test

Answer 6

WBC >20 x 109/L with Sézary cells

Question 7

Sézary syndrome Origin

Answer 7

can be considered to have evolved from MF (not initially meeting diagnostic criteria), but more
commonly arises de novo

Question 8

Sézary syndrome prognosis

Answer 8

often fatal

Question 9

CTCL Pathophysiology

Answer 9

clonal proliferation of skin-homing CD4 T-cells

Question 10

CTCL Epidemiology

Answer 10

> 50 yr old, M:F 2:1

Question 11

CTCL Differential Diagnosis

Answer 11

tinea corporis, nummular dermatitis, psoriasis, DLE, Bowen’s disease, adult T-Cell leukemia-lymphoma
(ATL)

Question 12

CTCL Investigations

Answer 12

skin biopsy (histology, “lymphocyte antigen cell” markers, TcR gene arrangement)
• blood smear looking for Sézary cells or ow cytometry (e.g. CD4:CD8 >10 is Sézary)
• imaging (for systemic involvement)

Question 13

Mycosis fungoides Management

Answer 13

depends on stage of disease

topical steroids and/or PUVA, NB-UVB (311-313 mm)

Question 14

Sézary syndrome Management ORAL and first

Answer 14

oral retinoids and IFN

Question 15

Sézary syndrome Management Therapies

Answer 15

extra-corporeal photopheresis
may need radiotherapy for total skin electron beam radiation
may maintain on UV therapy

Question 16

Sézary syndrome Management Other therapies

Answer 16

other chemotherapy agents