Cutaneous T-cell lymphoma

Synonyms

1

Overview

Cutaneous T-cell 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 as non-Hodgkin's lymphomas. The most common types are mycosis fungoides and Sézary syndrome. In 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. The specific signs and symptoms vary from person to person. The exact cause of these conditions is unknown.

Symptoms

The stage of CTCL indicates how the disease is affecting the skin. There are three recognisable stages of early CTCL, although the disease will not necessarily progress through all three stages.

  • Premycotic or pre-tumour stage: Small, raised, red patches appear on the skin – commonly on the breast and buttocks, although they can appear anywhere. At this stage the disease often looks like common skin conditions such as eczema or psoriasis.
  • Plaque or infiltrative stage: Irregularly shaped red patches (plaques) form. Any part of the body may be affected, but the buttocks, skin folds and face are particularly common places. There may be permanent hair loss from the affected areas if the plaques are left untreated.
  • Tumour stage: Only a small proportion of people progress to this stage (most people never progress beyond the first stage). Raised lumps (tumours) appear on the skin. The tumours and the red plaques may become deep sores (ulcerate). At this stage the cancer may also have affected the lymph nodes. Rarely, it will affect major internal organs such as the liver, lungs, and spleen.

Sezary syndrome CTCL is referred to as Sezary syndrome when large areas of the skin are affected and large numbers of abnormal lymphocytes are also found in the blood. In some people there are no plaques or tumours, but the whole skin can be red, thickened, swollen, and sore (known as l’homme rouge). The skin may also be itchy and the surface may peel off.

Causes

The causes of CTCL are unknown.

Prevention

Skin moisturising creams can reduce skin discomfort. Other preparations can be added to bath water to keep the skin supple and relieve irritation Your doctor may prescribe steroid creams or ointments that can help the skin to heal.

Diagnosis

Dermatologists work with hematologists, pathologists, radiologists and other experts to diagnose cutaneous T-cell lymphoma. Tests and procedures used to confirm a diagnosis include:

  • Physical exam. Your doctor will examine your skin for patchy, scaly regions or solid, raised growths. You will also be examined for signs that your lymph nodes or other organs might be affected.
  • Blood tests. Your blood may be tested to determine if it contains skin lymphoma cells. Doctors may use advanced laboratory tests to analyze your blood in order to better understand your condition, including flow cytometry and polymerase chain reaction testing.
  • Skin biopsies. A pathologist examines a small sample of skin removed during a biopsy to determine whether it contains cancer cells. Sometimes multiple skin biopsies are necessary in order to confirm your diagnosis. Advanced laboratory tests, such as immunohistochemistry and polymerase chain reaction, may be used to help your doctor understand your prognosis and select the most effective treatment.
  • Imaging tests. Imaging tests, such as computerized tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET), can help determine if cancer cells have spread to other areas of the body.

Prognosis

CTCL is a low-grade lymphoma and usually develops very slowly. It may be many years before it develops from one stage to the next. Most people never progress beyond the first stage. Some people do not experience the first two stages; the appearance of a tumour (stage 3) can be the first sign of their disease.

Treatment

Approved treatments:

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

Resources

Refer to Research Publications.