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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
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Objectives 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
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Abnormal growth of T-lymphocytes (a type of blood cells) in the skin
CTCL Abnormal growth of T-lymphocytes (a type of blood cells) in the skin
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CTCL
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CTCL
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(Majority of patients)
Skin lesions patches of erythema and scaling Slightly raised plaques (Majority of patients)
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Skin lesions Tumors
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Skin lesions Erythroderma
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Other skin presentations
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The role of the dermatologist
Making the diagnosis: History and physical examination Skin biopsy(ies) Workup (staging): Blood work Radiological studies (if required):CXR, US, CT Treatment
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Why does it take so long to diagnose MF?
On average it takes 3 years from the development of skin lesions It is a rare disease It mimics other common skin diseases It could be asymptomatic limited disease Even if suspected, the skin biopsies might not be diagnostic 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
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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
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“Rarely progresses, frequently relapses”
Treatment options “Rarely progresses, frequently relapses” Active observation Topical agents: Topical steroids: symptomatic lesions Imiquimod (Aldara): localized lesions Topical Retinoids (Tazarotene): localized lesions Intralesional steroids
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Aldara reaction
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Phototherapy 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
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Systemic Isotretinoin
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
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Steps to manage the itch
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
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St Johns Institute of Dermatology
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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:
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