T-cell lymphomas: types, symptoms, diagnosis, treatment and prognosis

T-cell lymphomas are most often diagnosed in patients in old age, but adolescents and children are also susceptible to this disease. And men suffer them much more often than women.

What is T-cell lymphoma?

Lymphoma is a malignant lesion of lymphoid tissue and other organs. In the case of T-cell lymphoma, the disease affects the skin and lymph nodes (that is, it has epidermotropic character). The main elements that participate in the process of the formation of this tumor are T-lymphocytes (cells that normally provide an adequate immune response). T-cell lymphoma (pictured below) can be formed as a result of accumulation of pathological cellular elements in tissues.

Causes and predisposing factors

• Heredity
• Immunodeficiencies.
• Prolonged exposure to adverse factors: ultraviolet radiation, radiation, aggressive chemical agents.
• Age aspect (elderly patients).
• The presence of viruses (HIV, Epstein-Barr, hepatitis C, B, T-lymphocyte virus) in the body, intestinal diseases.
• The presence of mutations in the immune system, the transplantation of organs and tissues, the appointment of immunosuppressive therapy.

All of the above factors can worsen (respectively, provoke the development of the disease), stress, malnutrition, nervous overload, non-observance of rest and labor.

Classification

• T-cell lymphoma of the skin. Such lymphomas, as a rule, develop as a result of various mutations of T-lymphocytes, leading to uncontrolled reproduction of the latter and, as a result, their spread in the layer of the epidermis. This type of lymphomas is characterized by the appearance of polymorphic rash (plaques, tumors, spots, blisters). And in the beginning, patients complain of the emergence of flaky or itchy spots, eventually transformed into plaques, and subsequently into the foci of the tumor.
• Peripheral T-cell lymphoma. This concept includes all lymphomas that have a T-cell or NK-cell origin (except for new growths from immature T cells and T-lymphoblastic leukemia). Quite often, such lymphomas occur with the damage of blood, skin, bone marrow and internal organs, and diffuse infiltrative changes are observed in the lymph nodes.
• Angioimmunoblastic T-cell lymphoma. It manifests itself in the form of lymphoid tissue consolidation (in the course of histological examination of lymph nodes) by immunoblasts and plasma cells, with further complete transformation of its structure and the formation of pathological blood vessels.
• T-cell lymphoblastic lymphoma. New education, which is based on pathological changes in T cells. T-lymphocytes do not ripen, their nucleus takes on an irregular shape, they quickly and uncontrollably divide. This type of lymphomas is rare. When a similar pattern occurs, differential diagnosis is performed with acute leukemia.
• Non-Hodgkin's T-cell lymphoma. It represents a large group of neoplasms that consist of mature T-lymphocytes and do not belong to the lymphogranulomatosis (that is, Hodgkin's disease). Belonging to this group is determined by histological examination (in case of limphogranulomatosis, specific cell elements of Berezovsky-Shtenberg are found in the preparation, in the case of non-Hodgkin's lymphomas they are not).

Stages of development

1. The defeat of one lymph node.
2. The spread of the tumor process to two lymph nodes, which are located on one side of the diaphragm.
3. Multiple lesions of lymphatic structures on either side of the diaphragm.
4. The defeat of lymph nodes and internal organs, the presence of metastases in the kidneys, bone marrow, liver or organs of the gastrointestinal tract.

General symptomatology

The presence or absence of certain clinical manifestations depends on the type of tumor and its location. However, there are symptoms that are inherent in almost all T-cell lymphomas:

• Sweating at night.
• Rapid, causeless weight loss.
• Mushroomed mycosis.
• Subfebrile body temperature.
• Frequent weakness and apathy.

Mycosis mushroom

Mushroom mycosis is one of the most common manifestations that characterizes T-cell lymphomas. It occurs in seventy percent of patients with this disease and has three forms: classical, decapitated and erythrodermic.

Classical fungal mycosis passes through three stages of development:

• Erythematous - according to clinical signs similar to psoriasis, eczema and so on. Patients notice the presence of spots of oval or round shape, with clear boundaries, various sizes, having a pink-violet color and flaking on the surface. Such elements are more often located on the face and trunk, gradually increasing in quantity and passing to other areas. Such rashes can not be treated and may not last a long time or suddenly disappear. In addition, the spots can be combined with an itch, which is not stopped by conventional means.
• Plaque-infiltrative - occurs a few years after the onset of the disease. In place of the above-described spots, dense plaques with rounded or non-correct, but clear boundaries, are of purple color with peeling on the surface. On the site of the disappeared plaques are brown areas, or atrophy of the skin. The itching becomes worse, it becomes unbearable. There is rapid weight loss and fever, lymphadenopathy can be present.
• Tumor - in the place of plaques or on the unchanged skin, dense red-yellow, painless, tumor-like formations appear spherical or slightly flattened, resembling a mushroom cap. The diameter of such formations varies from one to twenty millimeters, and the number can be different. When the tumor breaks down, painful ulcers develop in its place, the depth of which can reach the fasciae or bones. The spleen, lymph nodes, lungs and liver are most often affected. Intoxication and weakness develops. The patients are suffering from heart failure, amyloidosis, pneumonia.

Erythrodermic form is characterized by the onset of excruciating painful itching, hyperemia and swelling, the appearance of confluent erythematous-squamous spots, hyperkeratosis on the soles and palms, and hair loss. All lymph nodes in axillary, inguinal, cubital and femoral zones are enlarged. With their palpation, dense, painless lymph node packs that are not soldered to the surrounding tissues (skin) are found. There is a gout (up to 39 degrees), weight loss, night sweats and weakness. Lethal outcome is possible with aggravation of intoxication and adherence to heart failure.

In the presence of a headless form on a healthy skin, multiple plaques develop, eventually acquiring a malignant nature. Death of patients occurs within a year.

Clinical manifestations of cutaneous form of lymphomas

T-cell lymphoma of the skin often arises against the background of long-existing and improperly treated dermatological diseases. To provoke her appearances can herpes viruses, radiation, sunburn and so on. There are similar lymphomas in two stages: primary (the process is localized in the dermis), secondary (when the skin lesions are a consequence of the penetration of lymphocytes from the internal organs).

The main symptoms are:

• Polymorphic skin rash.
• Itching skin itch.
• The presence of small flat elements with a knot form.
• The appearance of yellowish plaques, which subsequently transform into foci of hyperpigmentation and atrophy (with plaque form).
• A clinical picture resembling psoriasis (with a shallow-necked type).
• Dryness, swelling, hyperemia and peeling of skin areas (with erythrodermic type of disease).

Symptomatic of peripheral lymphomas

Clinic of the disease depends on the stage and type of pathological process.

Characteristic increase in lymphoid tissue in the cervical, inguinal and axillary areas.

Patients complain of heavy sweating, severe weight loss, lack of appetite, general weakness, fever, and in the presence of an increase in the spleen and liver (which is often enough) - a shortness of breath, a periodic cough and a feeling of heaviness in the stomach.

Diagnostics

• Inspection, collection of complaints and anamnesis in an oncologist.
• General analysis of blood and urine.
• Biochemistry of blood, as well as analysis for antibodies to hepatitis viruses.
• Biopsy of the damaged lymph node (which allows verifying the diagnosis of cell lymphomas).
• Ultrasound.
• MRI.
• CT.
• Oncoscreening of the body.
• Immunophenotyped study.
• Molecular-genetic analyzes.
• Cytogenetic analysis.

Therapy

Treatment of T-cell lymphoma depends on the form and stage of the process.

Thus, for example, for the erythematous stage of fungal mycosis, special antitumor therapy is not prescribed, but external corticosteroids and interferons are used.

Antitumor treatment is reduced to chemotherapy and radiation therapy.

In accordance with the form of the disease (indolent, aggressive and highly aggressive), the duration and composition of treatment, as well as rehabilitation, is determined.

Indolent lymphomas do not heal, but are only regularly observed if the process progresses, the tactics are changed.

In the treatment of aggressive forms, a combination of chemotherapeutic drugs and Rituximab (monoclonal antibodies) is used.

When it comes to highly aggressive lymphomas, prescribe drugs that are used in the therapy of acute leukemia.

Quite often chemotherapy is combined with stem cell transplantation.

However, the most effective treatment for lymphoma is a 30-day course of radiotherapy (according to the method of Elekt Sinerzhi), and after taking chemotherapeutic drugs.

Complications of chemotherapeutic agents can be: lung cancer, breast cancer and so on. Radiation therapy can be complicated by the development of coronary atherosclerosis.

After successful treatment, mandatory rehabilitation is carried out.

T-cell lymphoma: prognosis

• With the timely detection of the disease (the initial stage) and the appointment of an adequate treatment, the five-year survival rate is eighty-five percent, and for patients of child age - up to ninety percent.
• Regarding angioimmunoblastic lymphoma, the prognosis is disappointing. The average life expectancy in patients with a similar diagnosis is only two and a half to three years. Survival in the course of five years is observed only in a third of patients.
• In the presence of the T-lymphoblast form, the prognosis depends on the presence / absence of bone marrow damage. If the bone marrow is not affected, the outcome is usually favorable. Otherwise, only twenty percent of patients can expect improvement.
• The prognosis and survival with mushroom mycosis depends on the form and stage of the process. Thus, in the classical form, survival is up to ten years, and the prognosis is unfavorable in the case of pneumonia, and so on; The erythrodermal form is sporadic by the addition of various complications, as a result of which death occurs in two to five years; With a headless form, the forecast is unfavorable, death occurs in a year.