Mature T-cell lymphomas
Leukemia or T-cell lymphoma in adults.
What is it about?
T-cell lymphoma in adults is an aggressive type of T-cell lymphoma where cancerous T cells are found in peripheral blood flow.
T-cell leukemia/lymphoma is rare in North America and is more common in countries such as Japan and China, where a viral infection called HTLV-1 is more common. HTLV-1 infection increases the risk for people to develop this type of T-cell lymphoma.
This type of NHL can be declared at any age from early adulthood to advanced age. He/she is slightly more frequent in men than in women.
What are the symptoms?
Among the most common symptoms are: swollen lymph nodes, hypertrophied (lymphadenopathy) and an enlarged liver and spleen (hepatosplenomegaly).
During the examination, the patient may show signs that the skin and/or bones are affected, corresponding to high levels of calcium in the blood (hypercalcemia) and high levels of an enzyme called lactate dehydrogenase (LDH).
How is it diagnosed?
The diagnosis of leukemia/T-cell lymphoma in adults is performed from a lymph node biopsy. A lymph node sample is taken, which is then observed under a microscope to see if there are cancer cells.
X-rays, bone marrow biopsy, scans and blood tests can also be performed.
The presence of the HTLV-1 virus can also be determined.
How do we treat it?
Adult T-cell leukemia/lymphoma is treated through combined chemotherapy regimens.
Some patients may live for a long time with this cancer.
Large cell anaplastic lymphoma (LALRG) The systemic type and the primitive skin type
What is it about?
Large cell anaplastic lymphoma may appear in two different forms:
A systemic form in which lymphoma is present throughout the body;
A primitive skin form where it only declares itself in the skin.
The term "anaplastic" refers to the appearance of lymphoid cells that have a rather different appearance than normal lymphocytes.
Patients with large cell anaplastic lymphoma are generally young, of an average age of 33 years, and 70% of patients are male.
Anaplastic lymphoma with large cells of a systemic type is part of the aggressive NHL while the primary skin type is indolent.
The cancer cell of the two types of large cell anaplastic lymphomas can be either a T cell or a cell that does not have B cell markers or T cells (called "zero").
What are the symptoms?
Anaplastic lymphoma with large cells of a systemic type:
Patients with anaplastic lymphoma with large systemic-type cells usually have swollen or hypertrophied lymph nodes and others of their organs are affected.
Systemic symptoms and high levels of an enzyme called lactate dehydrogenase (LDH) occur in about 50% of patients. The bone marrow and the gastrointestinal tract are rarely affected, unlike the skin, which is frequently affected.
Anaplastic lymphoma with large cells of the primitive skin type:
Patients with anaplastic lymphoma with large cells of the primary skin type usually have a single lump or ulcerative tumor on the skin. Lymph nodes in the affected area may also be affected.
The cells affected by this type of lymphoma have a certain protein on the surface, called the CD30 antigen.
Patients can see spontaneous remissions with this disease. However, remissions are inevitably followed by recurrences.
There is a benign state that may resemble a anaplastic lymphoma with large cells of the primitive skin type and bears the name Lymphomatoid papulosis.
How is it diagnosed?
Both types of large-celled anaplastic lymphomas are diagnosed from tumor tissue biopsy. A sample of the tumor is taken, which is then observed under a microscope. The specific characteristics of the disease are identified and the diagnosis is established.
How do we treat it?
Anaplastic lymphoma with large cells of a systemic type:
For patients with this lymphoma, the patterns used in the treatment of other aggressive lymphomas, such as diffuse lymphoma with large B cells, are generally employed. Including:
CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone), a standard therapy used to treat anaplastic lymphoma with large cells of a systemic type;
Other treatments such as radiation therapy, stem cell transplantation and steroid therapies.
With combined chemotherapy, many patients with large cell anaplastic lymphoma will be cured.
Anaplastic lymphoma with large cells of the primitive skin type:
As mentioned, spontaneous remission is possible with this disease. If no remission occurs, the most common treatments are:
Radiation therapy in the affected area
Surgery to remove the affected skin area
Systemic chemotherapy (only used in patients with extensive illness and cannot be treated with local treatments).
T-cell cutaneous lymphoma (LCCT)
What is it about?
LCCT is a rare NHL type caused by cancerous growth of T cells in the skin. It is more common in adults, aged 40 to 60 years.
There are a few subcategories of LCCT, the most common being:
Sezary syndrome: It is a specific type of LCCT that affects large skin regions or lymph nodes. Patients with this lymphoma may have redness on the entire surface of their skin and cancer cells that move through the bloodstream. This type of LCCT often evolves aggressively.
Fungoides fungus: This is the name that is generally given to other types of LCCT when blood is not affected. This is a type of painless lymphoma where patients often had skin problems that could be akin to eczema for several years, before the diagnosis was finally established. In the early stages of the disease, biopsies may be difficult to interpret and the correct diagnosis can only be made after observing the patient for a long period of time. In advanced stages, lymphone can propagate to the lymph nodes and other organs.
What are the symptoms?
The LCCT can be manifested by small, prominent red plaques on the skin, often on the chest, buttocks, skin folds and face. These plaques often resemble eczema or psoriasis and may be associated with falling hair or bristles in the area concerned.
Patients who are at an advanced stage may have ulcerative tumors on the skin.
Lymph nodes in the affected area may also be affected.
How is it diagnosed?
The diagnosis is based on a skin biopsy of the affected skin region. A small sample of skin is taken from the affected area and then observed under a microscope.
How do we treat it?
Many treatments are used to treat LCCT. Including:
PUVA Therapy: It is also called photochemotherapy. This treatment is used when large areas of the skin are affected. PUVA therapy is performed using a drug called Psoralen and Ultraviolet A (UVA) light. The psoralen makes the skin more sensitive to the salutary effects of UVA light. This treatment is equivalent to sitting under an ultraviolet light and can be prescribed several times a week.
UVB therapy: Ultraviolet B light (UVB) slows the growth of cancer cells in the skin. This treatment does not include taking a medication that makes the skin more sensitive. Treatment can be prescribed several times a week.
Radiotherapy: Local radiation can be used for patients with LCCT and are at an early stage, when one or two small areas of skin are affected. Radiotherapy can also be used to treat the entire surface of the skin if the LCCT is wider. This type of radiation treatment is given the total électronothérapie name of the skin. It can only be prescribed once and can then be followed by several PUVA therapies if necessary.
Chemotherapy: Chemotherapy medications can be applied directly to the skin in the form of ointment. It is imperative that you follow the instructions carefully and apply the cream only the number of times indicated. You may also use intravenous chemotherapy if you are suffering from a LCCT at a more advanced stage.
The Bexarotene (TargretinMD): This is a drug that can be used to treat cases of advanced LCCT. It comes in the form of a capsule to be taken once a day during the meal.
Interferon: Interferon is a protein that is naturally present in the body. It is an important part of a healthy immune system. An artificial form of interferon can be injected under the skin (subcutaneously) to try to stimulate the immune response and fight the LCCT.
Photopheresis trials: This treatment is used in particular for Sezary syndrome. The patient's blood must pass through a machine where it is exposed to ultraviolet light.
Peripheral T-Lymphomas:
What is it about?
They are an aggressive NHL group that affects a certain type of T-cell. They account for about 7% of all NHL cases.
There are many subcategories of peripheral T-lymphomas. Including:
Subcutaneous T lymphoma to panniculitis type: This type of peripheral T lymphoma is quite rare and is often mistaken for a disease called panniculitis, an inflammation of the body's adipose tissue. The most common symptoms are the appearance of nodules under the skin (subcutaneous nodules) that can progress in open and irritated wounds. Hémophagocytaire syndrome, a serious disease in which parts of the immune system are activated uncontrolledly, is also common with this cancer.
Hepatosplenic Gamma-Delta lymphoma: This type of peripheral T lymphoma is a systemic disease (which affects the whole body) that is manifested by the infiltration of cancerous T cells into the liver, spleen and bone marrow. As a rule, there are no real tumors. It is associated with systemic symptoms (e.g., fever, weight loss, night sweats, fatigue) and is quite difficult to diagnose.
Entéropathique intestinal T-type lymphoma: This type of peripheral T lymphoma is very rare and it manifests in patients who suffer from an intestinal disease linked to gluten intolerance that has not been treated and is called celiac disease . Patients are often in a very weakened state and may suffer from intestinal perforation (an abnormal hole in the wall of the intestine).
Nasal extraganglionnaire T-type lymphoma: This type of peripheral T lymphoma, previously described as Angiocentrique lymphoma, is more common in Asia and South America than in North America and Europe. It affects the nose and nasal passages more frequently, but can also affect other organs. It evolves aggressively and patients are frequently afflicted with Hémophagocytaire syndrome, a serious disease in which parts of the immune system activate uncontrolledly.
T Angio-immunoblastic lymphoma: It is one of the most common subcategories of peripheral T lymphoma, representing about 20% of all T-cell lymphomas. Among the most common symptoms are: generalized lymphadenopathy (hypertrophied lymph nodes), fever, weight loss, rashes and high levels of antibody in the blood.
Unspecified peripheral T-lymphoma: unspecified peripheral T-lymphoma is the most frequent peripheral T-lymphoma subcategory in North America. It is a specific subcategory that includes all unclassifiable peripheral T-lymphomas. It is clear that this sub-category represents a heterogeneous group of diseases for which there is a lack of evidence to establish a more precise definition. Most patients with unspecified peripheral T lymphoma have affected lymph nodes. However, a number of extraganglionnaires sites may also be affected (e.g., liver, bone marrow, intestinal tract, skin).
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