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 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

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

CTCL

CTCL

(Majority of patients) Skin lesions patches of erythema and scaling Slightly raised plaques (Majority of patients)

Skin lesions Tumors

Skin lesions Erythroderma

Other skin presentations

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

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

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

“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

Aldara reaction

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

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

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

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

Resources Cutaneous Lymphoma Foundation: http://www.clfoundation.org/