Cutaneous T-cell lymphoma

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Cutaneous T-cell lymphoma
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Skin lesions, rash, itching
Complications	Infection, systemic spread
Onset	Typically in adulthood
Duration	Chronic
Types	Mycosis fungoides, Sézary syndrome
Causes	Unknown, possibly genetic and environmental factors
Risks	Age, gender, ethnicity
Diagnosis	Skin biopsy, blood tests, imaging studies
Differential diagnosis	Psoriasis, eczema, other lymphomas
Prevention	N/A
Treatment	Topical corticosteroids, phototherapy, chemotherapy, radiation therapy
Medication	N/A
Prognosis	Variable, depends on stage and type
Frequency	Rare
Deaths	N/A

Cutaneous T- lymphomas (CTCLs) are a group of disorders characterized by an abnormal accumulation of cancerous T-cells (a type of white blood cells ) in the skin resulting in an itchy, red rash that can thicken or form a tumor. CTCLs belong to a larger group of disorders known asnon-Hodgkin's lymphomas. The most common types are mycosis fungoides and Sézary syndrome. some cases, cancerous T-cells may spread to the lymph¬†nodes and eventually to other body tissues¬†and organs , potentially resulting in life-threatening complications.

Cause

The exact cause of these conditions is unknown.

Signs and symptoms

The specific signs and symptoms vary from person to person. The presentation depends if it is mycosis fungoides or Sézary syndrome, the most common, though not the only types. Among the symptoms for the aforementioned types are: enlarged lymph nodes, an enlarged liver and spleen, and non-specific dermatitis.

Diagnosis

A point-based algorithm for the diagnosis for early forms of cutaneous T cell lymphoma was proposed by The International Society for Cutaneous Lymphomas in 2005.

Treatment

Romidepsin

There is no cure for CTCL, but there are a variety of treatment options available and some CTCL patients are able to live normal lives with this cancer, although symptoms can be debilitating and painful, even in earlier stages. FDA approved treatments include the following:

• Romidepsin (Brand name: Istodax)Treatment of cutaneous T-cell lymphoma (CTCL) in patients who have received at least one prior systemic therapy.
• Denileukin diftitox (Brand name: Ontak)Treatment of patients with persistent or recurrent cutaneous T-cell lymphoma whose malignant cells express the CD25 component of the IL-2¬†receptor.
• Bexarotene (Brand name: Targretin (topical))Treatment of cutaneous manifestations of cutaneous T-cell lymphoma in patients who are refractory to at least one prior systemic therapy.

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NIH genetic and rare disease info

• NIH info on Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma is a rare disease.