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Leukemic and Lymphomatous Infiltrates of the Skin

»Define lymphoma.

Mycosis Fungoides

»Is there a lymphoma that begins in the skin?
»What type of lymphoma is mycosis fungoides?
»How common is mycosis fungoides?
»How does mycosis fungoides begin?
»What is parapsoriasis?
»What type of skin lesions are seen in patients with mycosis fungoides?
»Describe the three subtypes of mycosis fungoides.
»What is the TNM classification of mycosis fungoides?
»How is mycosis fungoides treated?
»Describe topical nitrogen mustard (HN2) therapy.
»If a patient develops allergic contact dermatitis to topical nitrogen mustard, does the treatment have to be permanently discontinued?
»Is photochemotherapy an effective treatment of mycosis fungoides?
»What are the major side effects of bexarotene in the treatment of patients with cutaneous T-cell lymphoma?
»How does one manage the side effects of bexarotene?
»Are interferons effective in treating mycosis fungoides?
»Is chemotherapy an effective treatment of mycosis fungoides?
»What is extracorporeal photophoresis?
»Are there any other FDA-approved treatments for cutaneous T-cell lymphoma?

Other Lymphomas and Leukemias

»Outline the Ann Arbor clinical staging system for Hodgkin’s disease.
»What is a Reed-Sternberg cell?
»What are the histologic classes of Hodgkin’s disease?
»Does Hodgkin’s disease occur in the skin?
»How are cells immunophenotyped? What does the CD nomenclature mean?
»What is lymphomatoid papulosis?
»Are CD30-positive cells specific for lymphomatoid papulosis?
»What is HTLV-1 virus? What is its significance?
»Can multiple myeloma present with skin lesions only?
»What is pseudolymphoma of the skin?
»Can B-cell lymphomas present with skin lesions?
»What is the most common type of leukemia in adults?
»Can leukemia present with specific skin lesions?
»What are some nonspecific skin lesions seen in patients with leukemia?

 
 
 

Are there any other FDA-approved treatments for cutaneous T-cell lymphoma?

Yes. Denileukin diftitox (Ontak) is interleukin-2 (IL-2) conjugated to diphtheria toxin. The drug is given intravenously only to patients whose malignant cells express CD25, the IL-2 receptor. The drug can be associated with significant toxicity, including capillary leak syndrome, acute hypersensitivity–type reactions, hypoalbuminemia, and hypotension. Flulike symptoms for several weeks following infusion are frequently noted. Partial responses are reported in 30% and complete responses in 10% of patients.

Histone deacetylase inhibitors (HDACi) inhibit the deacetylation of histone proteins associated with DNA and nonhistone proteins. The exact mechanism involving the treatment of CTCL is unknown, but DNA microarray studies show that HDACi affects the expression of numerous genes and proteins involved in cell proliferation, migration, and apoptosis. Oral Vorinostat (suberoylanilide hydroxamic acid) targets HDAC class 1 and class 2 HDAC enzymes and is FDA-approved for the treatment of refractory CTCL, with response rates of 24% to 30%. Ongoing studies are looking at HDACi used in combination with other therapies (phototherapy, ECP, and oral bexarotene).

Gardner JM, Evans KG, Musiek A, et al: Update on treatment of cutaneous T-cell lymphoma, Curr Opin Oncol 21(2):131–137, 2009.