OVERVIEW

What is primary cutaneous T-cell lymphoma?

Cutaneous T-cell lymphoma (CTCL) is a group of hematologic malignancies originating from T cells (a type of immune cell). It primarily affects adults and elderly patients, with skin manifestations such as patches, erythroderma, tumors, and accompanying itching.

The most common type is mycosis fungoides (MF). Other types include Sézary syndrome (SS), primary cutaneous CD30+ lymphoproliferative disorders, and subcutaneous panniculitis-like T-cell lymphoma.

Treatments include topical medications, phototherapy, immunomodulators, and retinoids. Severe cases may require chemotherapy.

What is mycosis fungoides in primary cutaneous T-cell lymphoma?

Mycosis fungoides (MF) is a slow-progressing T-cell lymphoma primarily characterized by various skin symptoms, such as patches or tumors (varying in size and color), hypopigmentation or hyperpigmentation, erythroderma, and hair loss. Severe itching may occur, affecting daily life. It can also involve lymph nodes, blood, and internal organs.

What is Sézary syndrome (SS) in primary cutaneous T-cell lymphoma?

Sézary syndrome is a type of cutaneous T-cell lymphoma presenting as widespread erythroderma with scaly, hardened rashes. Symptoms may also include hair loss, ectropion, and skin keratosis. A key feature is a significant increase in a specific cell type in peripheral blood.

Which departments should patients with primary cutaneous T-cell lymphoma visit?

Dermatology, Oncology.

Is primary cutaneous T-cell lymphoma common?

It is rare, with an incidence rate of less than 1 per 100,000.

Can children or young adults develop primary cutaneous T-cell lymphoma?

Pediatric cases are rare but do occur.

Which populations are more susceptible to primary cutaneous T-cell lymphoma?

• The peak age of onset for mycosis fungoides is 55–60 years, with a male-to-female ratio of 2:1.
• Sézary syndrome also predominantly affects the elderly and is rare in patients under 30. Males are more frequently affected than females.

SYMPTOMS

What are the manifestations of primary cutaneous T-cell lymphoma?

Mycosis fungoides can be divided into the patch stage, plaque stage, and tumor stage, progressing very slowly, often taking years or even decades.

• Early manifestations resemble eczema or dermatitis.
• The patch stage is characterized by erythema of varying sizes, accompanied by mild scaling, sometimes with varying degrees of atrophy, hypopigmentation, or hyperpigmentation, as well as telangiectasia.
• As the disease progresses, it gradually evolves into thickened reddish-brown plaques with scaling and gradually enlarging areas.
• The tumor stage presents with patches, plaques, nodules, ulcers, necrosis, etc., accompanied by lymph node involvement and damage to internal organs.

Where can lesions of primary cutaneous T-cell lymphoma appear?

Although they may occur anywhere on the body, even on the palms and soles, lesions are mostly confined to the swimsuit-covered area.

CAUSES

What are the causes of primary cutaneous T-cell lymphoma?

The exact cause is unclear. It is speculated to be related to infectious factors and genetic abnormalities.

Is primary cutaneous T-cell lymphoma hereditary?

There is no clear evidence suggesting a hereditary tendency.

Is primary cutaneous T-cell lymphoma contagious?

No.

DIAGNOSIS

What tests are needed for the diagnosis of primary cutaneous T-cell lymphoma?

A full-body skin examination, blood cell analysis, chest X-ray, etc. Some patients may undergo CT or PET/CT scans, lymph node biopsy, bone marrow biopsy, etc., depending on their condition.

Which diseases can primary cutaneous T-cell lymphoma be easily confused with?

Mycosis fungoides needs to be differentiated from various common skin diseases, such as eczema, psoriasis, and other cutaneous lymphomas.

TREATMENT

How is primary cutaneous T-cell lymphoma treated?

Taking mycosis fungoides, the most common type, as an example:

• For mild cases, topical medications such as corticosteroids and nitrogen mustard (which can be irritating) may be used alone;
• For slightly progressed cases, topical treatments are combined with phototherapy, immunomodulatory drugs (e.g., interferon), or retinoids;
• For severe cases involving internal organs, chemotherapy is required.

Do patients with primary cutaneous T-cell lymphoma need follow-up examinations?

Yes, regular follow-ups are necessary. Patients may be monitored every 1–2 months. If symptoms are well-controlled, follow-ups can be extended to every 6–12 months.

What is the life expectancy for patients with primary cutaneous T-cell lymphoma?

Staging the disease helps predict survival outcomes:

• Patients with limited skin patches (<10%) have a favorable prognosis, with a lifespan similar to that of the general population;
• Patients with widespread skin patches but no organ involvement have a good prognosis, with an expected survival of over 10 years, though about 25% may experience disease progression;
• Patients with skin tumors or extensive erythroderma but no extracutaneous lesions have a median survival of around 5 years, with most eventually succumbing to the disease;
• Patients with lymph node or visceral involvement typically have poor outcomes despite treatment.

DIET & LIFESTYLE

How likely is primary cutaneous T-cell lymphoma to recur? How to prevent recurrence?

This is a chronic disease with a course that can last over a decade or even longer. During this period, inappropriate treatment or discontinuation of therapy may lead to disease recurrence.

To avoid relapse and delay disease progression as much as possible, active intervention and regular follow-ups should be initiated after diagnosis. Do not rely on so-called folk remedies or unverified traditional treatments, as there is currently no scientific evidence supporting the efficacy of Chinese herbal medicine for this disease.

What should patients with primary cutaneous T-cell lymphoma pay attention to in daily life and diet?

No special precautions are necessary. Maintain a balanced diet, engage in moderate exercise, and get adequate rest.

PREVENTION

Is there any way to prevent primary cutaneous T-cell lymphoma?

The cause is unknown, and there are no effective prevention methods.