Cutaneous T-cell Lymphomas (CTCLs)

What are Cutaneous T-cell lymphomas (CTCLs)?

Cutaneous T-cell lymphomas (CTCLs) are a group of disorders characterized by abnormal accumulation of malignant T-cells in the skin, which may result in the development of rashes, plaques, and tumors. CTCLs belong to a larger group of disorders known as non-Hodgkin’s lymphomas (NHLs). SUbdividions of CTCLs include:

• Granulomatous Slack Skin
• Lymphomatoid Papulosis
• Mycosis Fungoides
• Pagetoid Reticulosis (Woringer-Kolopp Disease)
• Primary Cutaneous Anaplastic Large Cell Lymphomas
• Sézary Syndrome
• Subcutaneous Panniculitic T-Cell Lymphoma

What are the symptoms of Cutaneous T-cell lymphomas (CTCLs)?

The signs and symptoms of CTCLs vary depending on the type. Most patients with CTCL experience only skin symptoms, without serious complications; however, approximately 10 percent of those who progress to later stages develop serious complications. The two most common types are mycosis fungoides and Sézary syndrome. Classic mycosis fungoides is divided into the following 3 stages:

• Patch (atrophic or nonatrophic): Nonspecific dermatitis, patches on lower buttocks
• Plaque: Intensely pruritic plaques, lymphadenopathy
• Tumor: Prone to ulceration

Sézary syndrome is defined by erythroderma and leukemia. Signs and symptoms include:

• Edematous skin
• Lymphadenopathy Palmar and/or plantar hyperkeratosis
• Alopecia
• Nail dystrophy
• Ectropion Hepatosplenomegaly may be present

What causes Cutaneous T-cell lymphomas (CTCLs)?

The exact cause of cutaneous T-cell lymphomas is unknown.

How is Cutaneous T-cell lymphomas (CTCLs) diagnosed?

The diagnosis of cutaneous T-cell lymphomas is based upon a thorough clinical evaluation, detection of certain symptoms and physical findings, a detailed patient history, and a variety of specialized tests. Exams to confirm diagnosis include:

• Biopsies, including blood marrow biopsy
• Blood tests
• Imaging tests 

What are the treatments for Cutaneous T-cell lymphomas (CTCLs)?

A wide variety of treatment options exist for individuals with CTCL including topical chemotherapy, radiation therapy, photochemotherapy, vitamin A derivatives (retinoids), and chemotherapy. These treatments may be used alone or in varied combination. In 2006, the FDA approved vorinostat (Zolinza) for treating CTCL. Zolinza is intended to be used when the disease persists or returns after treatment by other medicines.

Where can I find out more about Cutaneous T-cell lymphomas (CTCLs)?

• National Organization of Rare Diseases (NORD)
• Lymphoma Research Foundation
• Medscape