Cutaneous T-cell lymphoma (CTCL) is a rare and difficult to diagnose malignant neoplasm of the lymphatic system. The disease is caused by the uncontrolled growth of T cells located in the skin's lymphatic system. Compared to "classic" systemic lymphomas, it has different symptoms. How do you recognize skin T-cell lymphoma? What is the treatment?

Cutaneous T-cell lymphoma (CTCL)is one of over 40 types of non-Hodgkin's lymphoma.1CTCL stands out from the common image of lymphomas because it develops primarily in the skin, not in the lymph nodes.

The source of the neoplastic process in this type of cancer is the uncontrolled growth of T lymphocytes, i.e. cells responsible for cellular immune responses aimed at eliminating the "threat".

The primary tumor site for CTCL is the lymphocytes found in the skin, but lymphoma cells can sometimes spread through the lymphatic system to the lymph nodes through the lymphatic infiltrate and / or into internal organs.

The most common types of CTCL are:

  • mycosis fungoides (MF)
  • Lymphomatoid papulosis (very mild course of the disease)
  • primary cutaneous large T cell anaplastic lymphoma (pcALCL)2‚3‚4‚5

Fast and proper diagnosis and implementation of proper oncological treatment gives patients the opportunity to stabilize the process for many years and reduces the burden of living with the disease.

Non-Hodgkin's lymphoma (NHL) is the eighth cause of cancer in the world in men and eleventh in women. It is estimated that over 350,000 people are diagnosed with these lymphomas annually.6

Cutaneous T-cell lymphomas (CTCL) account for a total of 2-3% of NHL cases and 65-80% of all primary cutaneous lymphomas.1,2 ‚3‚4,5 ‚ 7‚8, 9,10,11,12

CTCL is considered a rare disease, i.e. according to the criteria of the European Medicines Agency (EMA), the number of people affected does not exceed 5 out of 10,000.13

According to the data possessed by prof. Małgorzata Sokołowska-Wojdyło, from the Department and Clinic of Dermatology, Venereology and Allergology, GUM, in Poland, about 2,000 people suffer from CTCL.

Mycosis fungoidesthe most common subtype of the disease accounts for approximately 60% of CTCL cases.

Primary cutaneous large T cell anaplastic lymphoma accounts for approximately 10% of cases.

Causes of cutaneous T cell lymphoma (CTCL)

As with many other neoplastic diseases, the causes of CTCL development are complex and not fully understood - the influence of genetic, environmental and immunological factors is indicated.2‚3,4

At the same time, attention is paid to conditions such as:

  • long-term occupational exposure to chemicals
  • occurrence of autoimmune diseases
  • atopic dermatitis
  • hives
  • taking immunosuppressive drugs
  • previous anti-cancer therapy

There are also reports of the impact of specific bacterial or viral infections.4,5,14,15

Note, however, that many patients diagnosed with CTCL may not have such factors at all.

T-cell cutaneous lymphoma (CTCL) - symptoms

CTCL has a different clinical course compared to "classic" systemic lymphomas. Most patients experience skin manifestations (skin eruptions, itching) from an early stage of the disease.

These signs are, however, non-specific and can often be confused with other skin conditions (e.g. atopic dermatitis, psoriasis or allergic reactions).4,11,14,15,16, 17

In the later stages of CTCL, the lymph nodes and other organs are invaded by lymphoma. In the advanced stages of the disease, the following occurrence is observed :

  • ulcerations
  • secondary bacterial infections on skin lesions
  • enlarged lymph nodes
  • as well as troublesome itching of the skin and pain4,16,17

The clinical course of the disease is slow and takes many years, therefore it is crucial to maintain the best quality of life for the patient.

Due to the very strong skin manifestation in the form of numerous erythematous-exfoliating lesions, infiltrative, blue-red tumors with a tendency to form massive ulcers, the disease very quickly leads to a feeling of lack of self-acceptance by patients and withdrawal from functioning in the environment.18,19,20

Studies also show that approximately 40% of CTCL patients experience pain, and 13% of CTCL patients experience little or no pain.

Patients, apart from itching, experience hair loss and other skin problems (oozing, skin infections, intense dry skin, peelingthin and sensitive skin), in addition, skin and muscle pain causing disturbances in sleep and movement, as well as difficulties with the functioning of the hand.

The listed symptoms obviously affect the physical functioning, but they are also a psychological burden and have a negative effect on the personal, social and professional life of patients.18,19

Due to illness, patients are forced to reduce their working hours or resign from work (retirement) as the disease progresses. The visibility of skin lesions also causes a sense of limitation and leads to increased skin coverage compared to he althy people.

Soreness and sensitivity of the skin also affect daily activities and constitute a significant barrier to normal functioning, resulting from the disfigurement of the body and the associated social stigmatization.18,19,21,22

According to an expertprof. Sokołowska-Wojdyło, Department and Clinics of Dermatology, Venereology and Allergology in Gdańsk

Establishing a CTCL diagnosis can be difficult

Often the symptoms of cancer can be similar to the skin changes in psoriasis or eczema. The harbinger of the most common skin lymphoma - mycosis fungoides - may be itching, as well as erythematous and infiltrative changes in areas not exposed to the sun's rays, sometimes erythroderma, i.e. generalized skin inflammation (the skin of almost the entire body is red). Most cutaneous lymphomas have many years of history. Some gradually progress, leading to painful tumors with disintegration, and in later stages to the involvement of lymph nodes and internal organs.

Cutaneous T cell lymphoma (CTCL) - diagnosis

In most cases of CTCL, the initial diagnosis is made by a dermatologist together with a pathologist.

The basic examination enabling the diagnosis of CTCL is the histopathological assessment of the skin, occasionally supplemented with the histopathological assessment of the lymph node or part of the affected organ.

If CTCL is suspected, an additional immunophenotypic assessment should be performed, which allows the lymphoma to be classified as a T, B or NK cell lymphoma.

  • Histopathological examination - what is it? The course and results of the histopathological examination

In doubtful situations, when the basic research panel does not give a clear diagnosis of CTCL, it can also be supplemented with auxiliary tests, e.g. molecular tests, imaging tests: X-ray - X-ray, computed tomography - CT.2, 3,4,5

  • Molecular diagnosticscancer

The diagnosis of CTCL is also supplemented with the assessment of the extent of skin lesions, their clinical advancement, as well as the assessment of factors influencing the prognosis.

Carrying out such a thorough diagnosis is crucial in selecting the appropriate method of therapy, as well as in assessing the effectiveness of the treatment.2,3,4,5,23

Cutaneous T-cell lymphoma (CTCL) - treatment

CTCL therapy depends on its subtype as well as the severity of the disease. Possible treatment options, depending on the CTCL subtype, include local treatment, phototherapy, radiotherapy, and in more advanced stages, systemic treatment.

An additional treatment option for young patients in good general condition in advanced stages of the disease is allogeneic stem cell transplantation (alloHSCT), which can be used after treatment failure.2

Category:

Oncology