Transcript Dermatology Aspects of Cutaneous Lymphomas

DERMATOLOGY ASPECTS OF
CUTANEOUS T-CELL
LYMPHOMA
Dr. Raed Alhusayen MD, FRCPC
Division of Dermatology
Sunnybrook Health Sciences Centre
Cutaneous Lymphoma Patient Education Forum
April 14, 2012
Objectives
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Brief description of cutaneous T-cell lymphoma
(CTCL): focus on Mycosis Fungoides
The role of the dermatologist in CTCL
Treatment options for early stage disease
CTCL
Abnormal growth of T-lymphocytes (a type of blood
cells) in the skin
CTCL
CTCL
Skin lesions
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patches of erythema
and scaling
Slightly raised plaques
(Majority of patients)
Skin lesions
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Tumors
Skin lesions
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Erythroderma
Other skin presentations
The role of the dermatologist
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a.
b.
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a.
b.
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Making the diagnosis:
History and physical examination
Skin biopsy(ies)
Workup (staging):
Blood work
Radiological studies (if required):CXR, US, CT
Treatment
Why does it take so long to diagnose
MF?
On average it takes 3 years from the development
of skin lesions
I.
It is a rare disease
II.
It mimics other common skin diseases
III.
It could be asymptomatic limited disease
IV.
Even if suspected, the skin biopsies might not be
diagnostic
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Multiple biopsies over a period of time might be
needed
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Staging of Mycosis Fungoides
MF stage
Description
IA
Patches & plaques < 10% BSA
IB
Patches & plaques ≥ 10% BSA
IIA
Patches & plaques + ENLARGED palpable
Lymph node
IIB
Tumors
IIIA
Erythroderma
IIIB
Erythroderma + Sezary cells > 5% (B1)
IVA
Sezary Syndrome
IVB
Lymph node involvement (pathology)
IVC
Metastasis
Staging of Mycosis Fungoides
MF stage
Description
IA
Patches & plaques < 10% BSA
IB
Patches & plaques ≥ 10% BSA
IIA
Patches & plaques + ENLARGED palpable
Lymph node
IIB
Tumors
IIIA
Erythroderma
IIIB
Erythroderma + Sezary cells > 5% (B1)
IVA
Sezary Syndrome
IVB
Lymph node involvement (pathology)
IVC
Metastasis
Treatment options
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I.
II.
III.
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“Rarely progresses, frequently relapses”
Active observation
Topical agents:
Topical steroids: symptomatic lesions
Imiquimod (Aldara): localized lesions
Topical Retinoids (Tazarotene): localized lesions
Intralesional steroids
Aldara reaction
Phototherapy
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Performed at PERC
More than 500 CTCL
patients (350 active)
NBUVB (3x/wk): very
effective on patches
and thin plaques, less
toxicity
PUVA (2x/wk): thicker
plaques, longer
remission
Systemic Isotretinoin
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Vitamin A derivative
Especially helpful when combined with
phototherapy
Very well tolerated at low doses
Does not suppress the immune system
TERATOGENIC
Need to monitor lipid profile and liver enzymes
Steps to manage the itch
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Bathing with lukewarm water followed by gently
patting the skin dry
Using moisturizers on regularly
Topical steroids
Oral antihistamines: Benadryl, Atarax, Doxepin
Low dose oral prednisone
St Johns Institute of Dermatology
Cutaneous lymphoma team
Multidisciplinary Team: Dermatologist / Clinical
Oncologist / Hematologist / NURSES
 50-60 patients (6-8 new)
 Overall similar treatment approach (bexarotene
notable exception)
Interesting ideas:
 Cutaneous lymphoma tumor board: reviewing all new
cases and selected follow ups
 Case manager: primary contact person for the patient
 Low dose prednisone for symptom relief
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Resources
Cutaneous Lymphoma Foundation:
http://www.clfoundation.org/
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