skin lymphoma | Cutaneous T-lymphoma

Cutaneous T-lymphoma

Cutaneous T-lymphoma (LTC) is a rare type of non-Hodgkin's lymphoma (NHL). The CTA is a cancer that originates in the mature T lymphocytes of the skin. Other types of T or B lymphocyte lymphoma may affect the skin, but they differ from the Cta.

The CTA usually occurs in adults between 50 and 60 years of age. It affects the man more than the woman.

Types of cutaneous T-lymphoma
The CTA does not represent a single disease but a set of different T-cell lymphomas that affect the skin.

The Mycosis fungoides is the most common form of LTC. It originates in the skin of areas of the body that are usually not exposed to the sun. It may appear as skin rashes or raised dented plaques (cupboards) scaly, red and itchy. It can be difficult to diagnose mycosis fungoides since it is likely to be confused with other skin problems. It evolves slowly (indolent), and the symptoms may be present for many months or years before it is diagnosed. The disease can evolve into tumors on the skin and eventually spread to the lymph nodes, liver, spleen, lungs or blood. In rare cases, the miosis fungoides can propagate to the bone marrow.

Sezary's syndrome is very rare. The doctors thought it was an advanced form of mycosis fungoides, but it's actually a different disease. Sezary syndrome evolves rapidly (aggressively). Lymphoma cells are present in the skin, blood and lymph nodes. Almost any or all of the skin is affected. It may look like sunburn, and it often happens that the skin is red, itchy and peeling. In the advanced stages of the disease, the skin can be very itchy, painful and cold-intolerant. The disease can also spread to other organs and cause other symptoms. The immune system of people with Sezary's syndrome is often weakened, thereby increasing their risk of infection.

Primitive cutaneous anaplastic lymphoma (ALCL) evolved slowly (indolent). It usually appears in the form of one or more masses in the skin. These tumors can be of different sizes and some may crack and become open lesions. The primary cutaneous ALCL is generally observed in the skin only. It can spread to nearby lymph nodes. It rarely spreads to other organs. The primitive cutaneous ALCL differs from the systemic primitive alcl that affects the skin.

Symptoms
The CTA can cause different types of skin problems. In the early stages, skin tumours may be confused with non-cancerous conditions such as psoriasis or eczema. The CTA may cause:

Small masses similar to buttons (papules);
Flat, thin and red plates;
Raised thick surfaces (cupboards);
Masses that can be felt at the touch (nodules or tumors);
Masses with open lesions.
Treatments
Different treatments can be used for the CTA. Some are localized, which means that they are only administered to the affected skin. Others are systemic, that is, they circulate throughout the body.

Ultraviolet light Therapy

For ultraviolet therapy, ultraviolet a (UVA) or ultraviolet B (UVB) rays are used to help destroy lymphoma cells. A special type of light is used to administer them.

In PUVA therapy, Psoralen is associated with UVA rays. Psoralen is a medicine taken in pill form. It is usually administered about 2 hours before treating the skin with UVA rays. Psoralen makes the skin very sensitive to the effects of UVA rays, which helps to destroy lymphoma cells. PUVA therapy sessions take place about 3 times a week until there is no longer a sign of LTC on the skin. Thereafter it is still administered but less often as maintenance treatment.

UVB phototherapy only uses UVB rays since it is not necessary to take a medication to make the skin more sensitive. It is often used to process thin plates of LTC. UVB Light therapy is usually administered 3 to 4 times a week until there is no longer a sign of LTC on the skin (about 30 to 40 sessions). Thereafter it is still administered but less often as maintenance treatment.

Radiotherapy

Only external radiotherapy can be administered or associated with other CTA treatments. Électronothérapie is often used, i.e., electrons emit radiation. Electrons do not cross the entire skin, so radiation does not affect other organs or bone marrow.

The local external radiation therapy is the administration of radiation to a small skin surface. It can be used if there is only one tumor or if there are thick tumors spread out on a small surface. It can also allow to treat plaques that remain after a PUVA therapy.

Total irradiation of the skin by electron beams (TSEB) is the administration of radiation to the skin of the whole body. It is used if the CTA is more extensive. In general, it is only administered once, but sometimes the treatment is repeated at lower doses if the CTA reappears after treatment. TSEB can cause a sunburn-like reaction and some people may lose their fingernails and toes as well as hair and hair.

Chemotherapy

Chemotherapeutic agents used to treat the CTA are applied directly to the skin (topical chemotherapy) in the form of ointment or liquid. They can also be administered as a systemic treatment, that is, circulating in the blood and destroying the cancer cells present throughout the body.

Topical chemotherapy is usually used to treat an early CTA that affects only small areas of skin (limited disease). The following topical chemotherapeutic agents may be used:

Mechlorethamine (nitrogen mustard, Mustargen)

Carmustine (BiCNU, BCNU)

Systemic chemotherapy is used to treat a more advanced CTA that has spread to other organs. It is also administered to treat a CTA that has reappeared (relapse) or is no longer responding to other treatments. The chemotherapeutic agents administered to treat the CTA are as follows:

Fludarabine (Fludara)

Cladribine (Leustatin)

Pentostatine (Deoxycoformycin, Nipent)

Doxorubicin liposomal doxorubicin (Caelyx)

Gemcitabine (Gemzar)

Methotrexate

Chlorambucil (Leukeran)

Etoposide (Vepesid, VP-16)

Temozolomide (Temodal)

Pralatrexate (PDX)

Vorinostat (Zolinza)

The following chemotherapeutic associations may be used to treat the CTA:

CHOP – Cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin), and prednisone

R-Chop – Chop and rituximab (Rituxan)

EPOCH – Etoposide, vincristine, doxorubicin, cyclophosphamide and prednisone

Biological therapy

Biological therapy stimulates, strengthens, restores or mimics the body's immune system to create a reaction against cancer cells. Biological medications such as interferon alpha (intron A, Wellferon) can be used to help strengthen the immune response and treat the CTA. Interferon alpha is injected into the fat underneath the skin using a needle. It can be administered alone or associated with other treatments, such as PUVA therapy.

Targeted treatment

Targeted treatment uses drugs to target specific molecules (such as proteins) located on the surface of cancer cells. These molecules contribute to the sending of signals that tell cells to grow or divide. By targeting these molecules, medications interrupt the growth and spread of cancer cells while limiting damage to normal cells. The targeted drugs administered to treat the CTA are among other things:

Rituximab (Rituxan)

Alemtuzumab (Campath)

Dénileukine Diftitox (Ontak), which is an association of interleukin-2 (Aldesleukin, Proleukin) and diphtheria toxin

Romidepsine (ISTODAX)

Photopheresis trials

Photopheresis trials is also referred to as an unbody photochemotherapy (PCE). It is a systemic treatment that is sometimes administered to treat sezary syndrome or a changing CTA.

The photopheresis trials resembles blood donation, except that blood goes into a particular device rather than a collector bag. The device removes blood lymphocytes, including lymphoma cells. These lymphocytes are then treated with psoralen and UVA rays. Once treated, they are mixed with the rest of the blood before returning them to the person. The procedure lasts a few hours. It usually takes place two days in a row. We'll repeat it in about four weeks.

Surgery

Sometimes we do surgery to treat the CTA. It is most often used to do a skin biopsy to diagnose the CTA. In some cases it can completely remove a tumor from the skin. Even if we do surgery to remove the tumor, we will use other treatments.

Stem cell transplant

Stem cell transplants are sometimes proposed to treat a CTA that does not respond to other treatments.