Cutaneous T-cell lymphoma

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at January 11, 2017
StartDiseasesCutaneous T-cell lymphoma

Cutaneous T-cell lymphoma, so-called because it attacks the skin, is a type of non-Hodgkin's lymphoma, which is a type of cancer of the immune system. This condition involves gene mutations of T cells. T cells are types of lymphocytes or white blood cells formed in the thymus, a gland at the base of the neck.

Contents

Definition & Facts

There are many forms of T-cell lymphoma, most broadly classifiable as indolent (slow-growing) or aggressive (fast-growing). Malignant T cells cause various lesions to occur, eventually forming plaques, tumors, and spreading to other parts of the body.

One of the most common T-cell lymphomas is cutaneous T-cell lymphoma, which can spread from the skin to the blood, the lymph nodes, and internal organs. Cutaneous T-cell lymphoma is usually indolent in early stages and only causes symptoms involving the skin. However, in some patients, it may metastasize and advance rapidly to lymph nodes or internal organs.

The two most common cutaneous T-cell lymphomas are mycosis fungoides and Sézary disease. Mycosis fungoides is the more prevalent of the two and constitutes about half of all cases. Some consider Sézary syndrome to be mycosis fungoides in an advanced, variant form while others consider it a separate disease altogether. Subcutaneous T-cell lymphoma is another form of cutaneous T-cell lymphoma, and it resembles panniculitis.

Symptoms & Complaints

Skin symptoms of mycosis fungoides appear as flat, scaly rashes; as plaques, which are thicker, raised, itchy and often mistaken for eczema, psoriasis, or dermatitis; and as tumors, raised bumps which may ulcerate. Some cases display more than one type of lesion.

Sézary syndrome involves lymphoma cells in the blood; thin, itchy rashes over most of the body; and patches and tumors in some cases. Changes in the nails, hair, or eyelids may occur, and lymph nodes may swell.

Causes

The underlying cause(s) of cutaneous T-cell lymphomas remain unknown. Researchers speculate that genetic factors, immunologic abnormalities, environmental toxicants, viral infections, bacterial infections, diet, and psychological stress may play various roles in causing this cancer.

Malignancies develop from abnormal changes in the structure and orientation of T-lymphocytes. Depending on the particular cancer, these abnormal genetic changes may be spontaneous and sporadic or, more seldom, inherited and congenital.

Suspected risk factors are immune systems weakened by organ transplants, human immunodeficiency virus (HIV) infection, and treatment for acquired immune deficiency syndrome (AIDS).

Diagnosis & Tests

A medical history, physical examination, and skin biopsy are the first essentials for diagnosis. Examination of the skin biopsy under a microscope searches for abnormal cells. The examiner checks the lymph nodes for swelling, and blood tests look for abnormal lymphocytes.

Some patients have computed tomography (CT) scans or magnetic resonance imaging (MRI)s of lymph nodes and organs to detect the extent of metastasis of the lymphoma. Scans are usually unnecessary for patients in the earliest stages of the disease. If the lymph nodes are swollen, there may be another biopsy to investigate it. Rarely, the physician may perform a bone marrow biopsy as an outpatient procedure.

Cancer staging describes how much skin the lymphoma has invaded and whether it has metastasized to the lymph nodes or other organs. Early stages of mycosis fungoides are difficult to diagnose because the symptoms and skin biopsy samples resemble those of other skin conditions. When diagnosed, most mycosis fungoides are in early (1 and 2A) stages, and many never grow beyond them. Mycosis fungoides has four main stages.

In Stage 1 the lymphoma affects only the skin. This stage has two substages: In Stage 1A the lymphoma affects less than 10 percent of the skin and more than that percentage in Stage 1B. In Stage 2A there are patches or plaques on the skin and lymph nodes swollen but no cancerous T cells yet present. In Stage 2B one or more tumors appear on the skin.

In Stage 3A more than 80 percent of the skin is sore and inflamed from the onset of erythrodermic mycosis fungoides; Stage 3B is similar and involves cancerous T cells (Sezary cells) in the blood.

Stage 4 has three substages: Stage 4A1, in which many cancerous T cells are in the blood (Sezary syndrome), Stage 4A2, in which many are in the lymph nodes, and Stage 4B, in which they have spread to body organs, the liver or spleen among others.

Treatment & Therapy

Topical therapies will generally be used for Stage 1 patients and systemic therapies or topical and systemic combinations for patients in Stage 2B. Treatment of mycosis fungoides usually consists of emollients (moisturizers) and antipruritics (anti-itch drugs). Mycosis fungoides treatment should be based on the stage and previous treatment. Patients will be advised to avoid long exposure to the sun.

Localized mycosis fungoides may benefit from radiotherapy, corticosteroids, or surgical excision. Extracorporeal photopheresis involves withdrawing blood from the patient and then re-infusing it into the body after it has been photosensitized and irradiated. This can be an effective treatment for Sézary syndrome and for erythrodermic mycosis fungoides. Bone marrow transplantation may be an important option for young patients with certain cases of mycosis fungoides as it reportedly has achieved complete clinical remission in even advanced stages.

The prognosis depends on the type: indolent or aggressive, how prevalent or widespread the lymphoma has become when discovered, and the patient's age and overall health. If diagnosis comes at an early stage, the prospects for effective long-term management and control of the disease with no apparent effect on life expectancy are good. Some patients go for long periods with no need for treatment. In some very early stages, treatment may be able to cure cutaneous T-cell lymphoma.

Prevention & Prophylaxis

The mysterious cause(s) of cutaneous T-cell lymphoma continue to be subjects of extensive medical research. Pending definitive conclusions, advanced diagnostic techniques enable physicians to detect cutaneous T-cell lymphoma early for improved responses to treatment, fewer side effects, and better outcomes.

Prophylactic measures for cancer generally stress healthy diets, regular vigorous exercise, and healthy lifestyles with no tobacco, limited consumption of alcohol, moderate use of salt, minimal use of refined sugar, and careful exposure to the sun.