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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?

 
 
 

How does mycosis fungoides begin?


Mycosis fungoides. A, A 12-year-old boy with extensive patch-stage mycosis fungoides. B, A patient with extensive patch-, plaque-, and tumor-stage mycosis fungoides. C, Tumor-stage mycosis fungoides. This patient had skin lesions for 18 years before tumors developed and a diagnosis was made.
Fig. 46.1 Mycosis fungoides. A, A 12-year-old boy with extensive patch-stage mycosis fungoides. B, A patient with extensive patch-, plaque-, and tumor-stage mycosis fungoides. C, Tumor-stage mycosis fungoides. This patient had skin lesions for 18 years before tumors developed and a diagnosis was made.
Typically, mycosis fungoides begins with persistent scaly patches (Fig. 46-1A) that respond poorly to topical therapy with emollients and topical steroids. In the early stages, skin biopsy is frequently not diagnostic. The average time from onset of skin lesions to diagnosis is 7 years. In this early phase of the disease, a diagnosis of parapsoriasis en plaque is often made. In time, the patches thicken and become plaques. Eventually, skin tumors develop (Fig. 46-1B,C) and the lymph nodes can become involved. Visceral disease is a late occurrence in this low-grade lymphoma. Median survival for persons with patchand plaque-stage disease is 12 years; for tumor-stage disease, it is 5 years; and for nodal or visceral disease, it is 3 years.

Epstein E, Levin D, Croft J, et al: Mycosis fungoides: survival, prognostic features, response to therapy, and autopsy findings, Medicine 51:61–72, 1972.