Advertising on the portal

Angioimmunoblastic t cell lymphoma. Characteristic features of T-cell lymphomas

T-cell lymphoma is a malignant disease characterized by the appearance of degenerated T-lymphocytes in the blood and is caused by a specific virus.

The main signs of the disease are damage to bone and muscle tissue, internal organs, and an increase in the concentration of calcium in the blood.

Lymphoblastic lymphoma is most often recorded in Japan, South America, Africa, and North America.

It has been revealed that men get sick more often. The oncological process develops mainly in adults.

Origin

It is known that the disease has a specific pathogen - T-cell virus type 1. After its penetration into the blood, human lymphocytes acquire the ability to reproduce unlimitedly. The role of hereditary factors and external carcinogens in the development of lymphoma cannot be ruled out.

Manifestations of the disease

T-cell lymphoma can occur in various forms: acute, lymphomatous, chronic, smoldering. General signs of this malignant process are:

  • peripheral lymphadenopathy, enlarged liver and spleen;
  • damage to the surface epithelium - a skin plaque, papule, tumor-like growth, ulceration is formed;
  • osteolysis – destruction of bone tissue.

Due to bone destruction, calcium levels in the blood increase. Later, secondary immunodeficiency develops, so patients die from infectious complications. There is also a chronic form, in which the number of atypical cells in the blood is low.

It is characterized mainly by skin lesions. Lymphatic collectors and central lymphoid organs are not affected. The prognosis for the chronic form is relatively favorable, since survival reaches several years.

Acute form

With this form of the disease, symptoms increase very quickly, the prodromal period is shortened to 2 weeks, and life expectancy after the first manifestation of lymphoma does not exceed 6 months. Statistical data show that such a course is recorded in 60% of patients.

Symptoms include skin changes, lung damage, bone destruction with hypercalcemia. Destruction of bone marrow elements is rare, so the blood picture is normal.

The skin of T-cell lymphoma looks like a fungal infection, so it is important to carry out a differential diagnosis. In the bones you can find foci of destruction that contain osteoclasts - cells that absorb bone beams, but there are practically no cancer cells there.

Foci of infiltration caused by the penetration of leukocytes and pneumonia caused by opportunistic microflora are detected in the lung tissue. Infectious complications have the same spectrum as with HIV infection.

A small percentage of patients have a specific complication – leptomeningitis. Its symptoms are headache, mental and sensitivity disorders. This disease is not accompanied by an increase in protein levels in the cerebrospinal fluid, which makes it possible to distinguish it from other lymphomas.

Lymphomatous form

It has a clinical course similar to the form described above, but in this case there is an enlargement of the lymph nodes. Observed in 20% of cases.

Chronic form

The chronic course is characterized by the preservation of the central nervous system, bones and gastrointestinal tract. Life expectancy is about 2 years. The transition from chronic to acute is possible.

Smoldering form

It is a rare type and accounts for about 5% of lymphoma cases. Cancer cells are found in the blood in small quantities. Symptoms of lung and skin damage may occur. The survival rate of patients is about 5 years.

Diagnostics

To make a diagnosis, a clinical examination is performed, general blood test (increased number of lymphocytes, atypical lymphocytes), skin biopsy (infiltration of the upper layers with cancer cells).

Oncologists also determine the level of calcium in the blood and conduct a test for antibodies to T-lymphotropic virus type 1 (molecular genetic study).

Therapy

Treatment of patients with T-cell lymphoma is complex. Since the tumor has low sensitivity to all methods of therapy, chemotherapy drugs are administered along with interferons, which significantly improves the result.

Prospects for recovery and prevention

Life expectancy depends on the level of calcium in the blood.

With normal indicators, the prognosis is relatively favorable - patients live about 50 months, with hypercalcemia - 12.5 months (2 weeks - 1 year). The cause of mortality is infectious complications and blood clotting disorders (DIC).

There are no specific methods for preventing lymphoma. People living in endemic areas where cases of the disease are common should be alert for lymphoma and seek prompt medical attention.

All iLive content is reviewed by medical experts to ensure it is as accurate and factual as possible.

We have strict sourcing guidelines and only link to reputable sites, academic research institutions and, where possible, proven medical studies. Please note that the numbers in parentheses (, etc.) are clickable links to such studies.

If you believe that any of our content is inaccurate, out of date, or otherwise questionable, please select it and press Ctrl + Enter.

Cancer is considered one of the most dangerous human diseases. And if you also take into account that under the terrible diagnosis there are several varieties of a fatal disease that have interrupted the lives of many people, then willy-nilly you will become interested in this issue in order to avoid a similar fate. Take, for example, one of the most dangerous types of blood cancer that affects the lymphatic system and skin, called T-cell lymphoma. It is because of this pathology that many elderly people do not live to see their long-awaited retirement or only enjoy it for a very short time. Although, to be honest, not only old people should be included in the risk group for developing this pathology.

ICD-10 code

C84 Peripheral and cutaneous T-cell lymphomas

Epidemiology

As for the statistics of lymphomas, T-cell lymphoma occupies a prominent place among cancerous blood pathologies observed in older age groups. However, it can hardly be called a disease of the elderly, because cases of the disease have been repeatedly recorded even in children and adolescents. Moreover, men are more predisposed to the disease than women.

The epidermotropic nature of the pathology suggests that the disease is localized mainly in the skin, organ membranes and lymph nodes located in close proximity to them. The most common form of the disease is T-cell lymphoma of the skin, and in particular mycosis fungoides.

, , , , , , , , , , , , ,

Causes of T-cell lymphoma

Lymphoma is a tumor neoplasm consisting of modified cells of the lymphatic system. In the case of T-cell lymphoma, T-cells (T-lymphocytes) that develop in the thymus gland from prethymocytes, which are contained in the red bone marrow, from where they migrate to the thymus, become participants in the pathological process.

T-lymphocytes are a factor that ensures the body’s immune response to various external influences and regulates the processes occurring inside it. So, any inflammatory process in the body causes a redistribution of T-lymphocytes, which accumulate near the lesion along the flow of lymph. After the inflammation is stopped, the lymph nodes regain their original size and elasticity. Alas, this does not always happen.

Scientists are not yet ready to give a full answer to the question of what causes the uncontrolled accumulation of lymphocytes at one point for no apparent reason, and what causes the mutation of cells of the lymphatic system, which begin increased division (proliferation), leading to unwanted proliferation of tissues inside the body. Uncontrolled proliferation of cells leads to an increase in the size of lymph nodes and organs into which mutated T-lymphocytes are supplied. All this causes dangerous disruptions in the functioning of organs and glands, leading to the death of the patient.

Most scientists are inclined to believe that T-cell lymphoma is a consequence of an unidentified leukemia (also known as leukemia). With leukemia, the appearance of modified cells formed from immature hemocytoblasts of the bone marrow, which are the precursors of blood cells, is observed. In T-cell leukemia, mutations of lymphoblasts and prothymocytes, the precursors of T-lymphocytes, are observed.

Such malignant clones from the red bone marrow can enter the thymus, and from it in the form of mutated T-lymphocytes prone to uncontrolled proliferation, and into the lymphatic system. Thus, T-cell leukemia type 1 (also known as HTLV 1 - Human T-lymphotropic virus 1), which belongs to the family of retroviruses, becomes the most likely cause of the development of T-cell lymphoma.

However, this theory does not explain the magnification of mature T lymphocytes, which were not initially pathogenic. It turns out that they experienced the negative influence of some factors other than the HTLV 1 virus, which led to mutations.

As one of the theories for the formation of T-cell lymphoma, the hypothesis about the negative impact of certain viruses on the quality of lymphocytes (for example, herpes viruses type 6 and type 4, hepatitis virus, HIV) is considered. These viruses are often found in patients with blood cancer, which means their influence cannot be ruled out.

Since the production of T-lymphocytes is carried out under the control of the immune system, any discrepancy in its work can lead to increased production of T-lymphocytes from bone marrow cells that do not have time to mature and a greater likelihood of chromosomal mutation in them. An inadequate response of the immune system to irritants can also manifest itself in the form of accumulation in the epithelial layers of the skin of rapidly dividing clones of lymphocytes, leading to the formation of microabscesses, which is observed in T-cell lymphoma of the skin.

Simultaneously with the proliferation of lymphocytes, there is a decrease in the activity of cells that provide antitumor protection.

Lymphocytes and some other cells in the body are capable of producing special information molecules called cytokines. These molecules, depending on their type, ensure the interaction of cells with each other, determine their lifespan, stimulate or inhibit cell growth, division, activity and death (apoptosis). They also ensure the coordinated functioning of the immune, nervous and endocrine glands. Among the cytokines there are molecules that inhibit inflammatory and tumor processes, and those that regulate the immune response. Reducing the activity of these molecules can pave the way for tumor formation.

, , , , ,

Risk factors

Risk factors for developing T-cell lymphoma include:

  • Inflammatory processes in the body that cause a corresponding reaction of the immune system and lead to the accumulation of lymphocytes in the affected area.
  • The presence of a viral infection in the body (herpes viruses type 1, 4 and 8, hepatitis B and C viruses, lymphocytic virus, HIV infection and even Halicobacter pylori).
  • Inadequate functioning of the immune system due to previously occurring mutations and structural changes. We are talking about autoimmune pathologies, the introduction of immunosuppressants into the body that inhibit the functioning of the immune system.
  • Prolonged contact with carcinogenic substances.
  • Hereditary predisposition and congenital immunodeficiency conditions.
  • Prolonged exposure to ionizing and ultraviolet radiation, certain chemicals that cause mutations in lymphocytes.
  • The chronic course of various types of dermatosis (psoriasis, atopic dermatitis, etc.), leading to prolonged presence of lymphocytes in the affected area, provoking a decrease in immunity and the proliferation of malignant clones in the local area. This can lead to the formation of a malignant process on the skin.
  • Advanced age.

Most often, the development of the disease is not caused by one cause, but by the combined influence of several factors. This may be why specific manifestations of the disease are observed mainly in adulthood, after the body has experienced the negative influence of many factors for a long time.

, , , , , , , , , , , ,

Symptoms of T-cell lymphoma

Speaking about the symptoms of the disease, you need to understand that the variety of types of T-cell lymphoma will leave its mark on the clinical picture of the disease in each specific case. However, there are some common signs that are characteristic of many cancer pathologies.

The first, albeit nonspecific, signs of the development of the disease are:

  • causeless loss of appetite, disruption of the digestive process,
  • weight loss, despite the fact that the amount and calorie content of food did not undergo significant changes,
  • chronic weakness, decreased performance,
  • apathy to what is happening around,
  • increased reaction to stress factors,
  • increased sweating (hyperhidrosis), especially at night,
  • constant slightly elevated temperature (low-grade temperature indicators are within 37-37.5 degrees),
  • chronic constipation, if the tumor is localized in the pelvic area,

Specific symptoms of T-cell lymphoma are:

  • persistent increase in size and hardening of lymph nodes,
  • an increase in the size of internal organs (most often the liver and spleen),
  • redness and burning of the skin, the appearance of rapidly growing lesions on it in the form of ulcers, plaques, papules,
  • disruption of the structure (destruction) of bone tissue.

Forms

The disease can have a different course, and therefore the following forms of pathology are distinguished:

  • Acute form.

It is characterized by rapid development of the disease. From the appearance of the first signs to the full disclosure of all symptoms, no more than 2 weeks pass. Such a course of the disease is observed in more than half of all patients diagnosed with T-cell lymphoma.

Enlarged lymph nodes are usually absent in this form of pathology. Death occurs within six months from the onset of the disease from complications in the form of pneumonia, infection in the affected areas of the skin, bone destruction with poisoning of the body with decay products, decreased immunity, leptomeningitis with mental disorders, etc.

  • Lymphomatous form.

Similar to the above in clinical picture. However, one of the symptoms of the pathology is enlarged lymph nodes. This course of the disease is observed in a fifth of patients.

  • Chronic form.

The clinical picture takes a longer time to emerge fully and is less pronounced. At the same time, the nervous, digestive and skeletal systems are not affected if the disease does not become acute. Patients live with the pathology for about 2 years.

  • Smoldering form.

The rarest form of the disease, characteristic of 5 patients out of 100. There is a small number of mutated T-lymphocytes, proliferation occurs more slowly than in other forms of lymphoma. However, symptoms of damage to the skin and lungs are noticeable. The life expectancy of patients is about 5 years.

We will talk in more detail about the manifestations of T-cell lymphoma, considering various types of pathology and the features of their course.

Since T-cell lymphomas can differ not only in the location of the pathological process, but also in external manifestations and mechanism of formation, it is customary to classify them into the following types:

The cause of such neoplasms is considered to be mutations of mature T-lymphocytes under the influence of unfavorable factors (viruses, radiation, burns), as a result of which they are able to actively reproduce and form clusters of mutated clones in the epidermal layer.

Clinical picture: the appearance of various rashes on the skin (plaques, blisters, spots, papules, etc.).

Pathology usually occurs in 3 stages. At the beginning of the disease, patients note the appearance of eczema-like spots that begin to itch and peel, then in their place plaque-like formations appear, gradually growing and rising above the surface of the body. Next, the “wrong” lymphocytes in the skin are joined by mutant clones circulating through the lymphatic system, the cancer metastasizes, which leads to the death of the patient within 2-5 years.

  • Peripheral T-cell lymphoma.

This concept includes all tumor elements formed by T- or NK-lymphocytes (anti-inflammatory elements and natural killer cells that provide an antitumor effect). Only mature cells prone to excessive proliferation are included in the process. Such lymphomas can form in the lymph nodes and on internal organs near the affected lymph nodes. They affect the quality of blood, the condition of the skin and bone marrow, and lead to the destruction of bone tissue.

Clinical picture: enlarged lymph nodes in the neck, groin and armpits. This type of pathology is characterized by the nonspecific symptoms of the disease described above, plus some unpleasant manifestations associated with enlarged organs (usually the liver and spleen). Patients note difficulty breathing, repeated coughing for no reason, and heaviness in the stomach.

Doctors rarely make a diagnosis of “peripheral lymphoma” if they cannot attribute the pathology to a specific type. Peripheral lymphoma is characterized by an acute (aggressive) course with the formation of metastases to other organs.

  • Angioimmunoblastic T-cell lymphoma.

Its characteristic feature is the formation of compactions in the lymph nodes with an infiltrate in the form of immunoblasts and plasma cells. In this case, the structure of the lymph node is erased, but a large number of pathological blood vessels form around it, provoking the appearance of new pathologies.

The disease has an acute course. Immediately, various groups of lymph nodes, liver and spleen enlarge, a rash appears on the body and other symptoms of cancer. Plasma cells can be found in the blood.

  • T cell lymphoblastic lymphoma, reminiscent of acute T-lymphoblastic leukemia in its course.

T-lymphocytes of irregular structure take part in the development of the tumor process. They do not have time to mature, therefore they have an imperfect nucleus, and therefore begin to quickly divide, forming the same structures of irregular shape.

This is a fairly rare pathology that has a fairly good prognosis if the disease is caught before it affects the bone marrow system.

T-cell lymphomas of various types usually go through 4 stages in their development:

  1. At the first stage of the pathology, there is an enlargement of only one lymph node or lymph nodes of one group.
  2. The second stage is characterized by an increase in lymph nodes of different groups located on one side of the diaphragm.
  3. The third stage of the disease is spoken of if the lymph nodes are enlarged on both sides of the diaphragm, which indicates the spread of the malignant process.
  4. The fourth stage is the spread of metastases. Cancer affects not only the lymphatic system and skin, but also spreads to internal organs (kidneys, lungs, liver, gastrointestinal tract, bone marrow, etc.).

Thus, the earlier lymphoma is detected, the greater the chance a person has of coping with the disease. At stage 4, these chances are practically reduced to zero.

Mediastinal T-cell lymphoma

The number of cancer diseases, sadly enough, tends to increase. Every year, oncology clinics are replenished with an increasing number of patients, among whom the lion's share of those diagnosed with mediastinal T-cell lymphoma.

For those who are not entirely familiar with medical terminology and human anatomy, let us explain that the mediastinum is not one of the organs, it is the area between the sternum and the spine where all the organs of the chest are located (esophagus, bronchi, lungs, heart, thymus, pleura, many nerves and vessels).

If uncontrolled division of T-lymphocytes leads to the development of a tumor on one of the chest organs, they speak of mediastinal lymphoma. Its danger lies in the fact that the process can quickly spread to other organs. After all, cancer cells calmly move through the lymphatic system, which washes various organs (including those affected by the disease) at least within one group of lymph nodes and beyond.

Symptoms of mediastinal lymphoma are generally characteristic of all oncological pathologies (weakness, nausea, weight loss, shortness of breath, etc.). A specific symptom indicating the localization of the pathological process is considered to be enlarged lymph nodes in and around the chest (neck, armpits, groin, abdomen). What is important is that when pressing on the swollen lymph nodes, the patient does not feel pain, which indicates not an inflammatory, but a malignant process in them.

In its course, T-lymphoma of the mediastinum goes through not even 4, but 5 stages:

  1. The appearance of several suspicious spots on the skin
  2. The number of skin formations increases, but the lymph nodes remain unchanged
  3. Swelling appears in the area of ​​the spots,
  4. The spots begin to inflame and turn red,
  5. The process spreads inward to the organs of the mediastinum.

T-cell lymphoma of the mediastinum is most often diagnosed in middle-aged and elderly people, and in the latter it is more severe, metastasizing quite quickly throughout the body.

, , , , , , ,

Diversity of T-cell lymphomas of the skin

A peculiarity of this type of lymphoma is that the uncontrolled proliferation of cells originates not in the lymph nodes and organs, but in the skin, from where the process spreads into the body. Among cutaneous T-cell lymphomas, there can be both fast-growing (aggressive) subtypes and varieties with an indolent course.

Doctors include aggressive lymphomas that quickly metastasize to other organs and glands:

  • Sézary syndrome, which belongs to the category of pathologies in which the level of eosinophils in the blood increases. In this case, 3 symptoms are necessarily present: erythroderma (redness of the skin with lamellar peeling, extensive red rashes), lymphadenopathy (enlarged lymph nodes) and the presence in the blood of specific cells with folded nuclei, which are detected during laboratory tests and biopsy.
  • Adult T-cell leukemia-lymphoma is a skin tumor caused by the retrovirus HTLV-1. Symptoms: damage to the skin and lymph nodes, enlargement of the liver and spleen (hepatosplenomegaly), complete, irreplaceable resorption of bone tissue due to metastasis (osteolysis).
  • Nasal type of extranodal T-cell lymphoma (polymorphic reticulosis). Develops from NK-like T. leukocytes of irregular shape. Affects the skin, upper respiratory tract (bronchi, trachea, lungs) and gastrointestinal tract, midline structures of the skull in the facial area (palate, nose, some parts of the central nervous system). Skin lesions in the form of compactions (plaques) of a brownish-blue color are observed already at stage 3 of the disease.
  • Peripheral unspecified T-cell lymphoma of the skin. It is characterized by the appearance of lesions in the epidermal layers and enlargement of the lymph nodes; subsequently the process can spread deep into the body.
  • Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma. Polymorphic elements (nodules, plaques, spots) form on the skin, often with ulcerations in the center. Infiltration can also be found in the lungs, testicles in men, mucous membranes, and the central nervous system.
  • Cutaneous γ/δ T is a cellular lymphoma in which necrotic nodules and tumors appear mainly on the extremities, sometimes on the mucous membranes. The bone marrow, lymph nodes and spleen are rarely affected.

This is an incomplete list of fast-growing lymphomas, the life expectancy of patients for which is no more than 2 years. Rapid development is more typical for peripheral lymphomas. Most likely this is due to the fact that the movement of lymph is directed from the periphery to the center, which means that “sick” leukocytes are quickly delivered to various internal organs, where they can settle and multiply rapidly.

A sluggish course of the process is observed in such pathologies as:

  • Mycosis fungoides, which many patients do not even associate with a cancerous tumor due to its similarity to some dermatological pathologies. Symptoms: the appearance of plaques of various shapes and sizes on the skin (sometimes covered with scales and itching, reminiscent of psoriasis), which gradually increase in size and turn into a tumor. In addition, swelling of the skin, enlarged lymph nodes (lymphadenopathy), thickening of the skin on the palms and soles (hyperkeratosis), hair loss, deterioration of the nails, swelling and inversion of the eyelids, enlargement of the liver and spleen are noted.
  • Primary anaplastic T-cell lymphoma, which is one of the subtypes of pathology with the general name “large cell lymphoma”. It is characterized by the appearance of purplish-red nodules of various sizes (1-10 cm) on the skin. These may be single rashes, but quite often the appearance of a large number of nodules is also observed.
  • Panniculitis-like subcutaneous T-cell lymphoma. The tumor process begins in the subcutaneous tissue. Symptoms: increased eosinophils in the blood, itching and rashes on the skin, constant fever, enlarged liver and spleen, enlarged lymph nodes, jaundice, edema syndrome, weight loss.
  • Primary cutaneous CD4+ pleomorphic T-cell lymphoma. The infiltrate consists of cells of small and large sizes. Rashes in the form of plaques and nodules can be seen on the face, neck and upper torso.

Complications and consequences

As for T-cell tumor pathologies, which belong to the category of malignant non-Hodgin lymphomas, it is not so much the pathology itself that is scary, but its complications. In the early stages, T-cell lymphoma affects only the skin and lymphatic system, resulting in minor illness. But an increase in tumor size and metastasis to other organs gradually disrupts the functioning of the entire body, which leads to the death of patients.

A greatly enlarged lymph node can compress the superior vena cava, which provides blood supply to the heart, esophagus and digestive tract, respiratory, urinary and biliary tracts, limiting the movement of liquids, air, and food through them. Compression of the spinal cord leads to pain and impaired sensitivity of the limbs, which affects their activity.

Cancer cells through the lymphatic system can spread to the bone, brain and spinal cord, bones, liver and other organs washed by lymph passing in the area of ​​one of the groups of lymph nodes. The tumor increases the size of the organ, reducing the internal volume or destroying the structure, which necessarily affects its functionality.

The disintegration of a large number of tumor cells leads to intoxication of the body with fever, weakness, and disruption of the joints due to the accumulation of uric acid in them (formed as a result of the decomposition of the cell nucleus).

Any oncological disease occurs against the background of a general decrease in immunity, which makes it possible for various types of infections (bacteria, viruses, fungus) to easily penetrate into the body. Thus, lymphoma can be complicated by infectious diseases.

The consequences of T-cell lymphomas depend on the degree of malignancy of the process, the speed of its spread, the accuracy of the diagnosis and the timeliness of treatment.

, , , , , , , , , , , , ,

Diagnosis of T-cell lymphoma

Despite the similarity between many types of T-cell lymphoma, the features of their course and approaches to their treatment may be different. This means that the doctor needs to be especially careful when diagnosing the disease in order to accurately determine its type, stage of development and develop a strategy to combat the terrible disease.

Diagnosis of T-cell lymphoma, as usual, begins with an examination by an oncologist. It is very important to tell your doctor about all the symptoms you have and when they appeared. This is necessary to accurately determine the stage of the disease and its prognosis. It is worth mentioning the cases of cancer in the family.

During the appointment, the doctor palpates the enlarged lymph nodes and determines the degree of their pain. In case of cancerous pathologies, the lymph nodes are painless.

The patient is then sent for tests. Initially, they give a referral for a blood test (general and biochemical) and a urine test, which can reveal the presence of inflammatory processes in the body, changes in the concentration of various blood components (for example, an increase in eosinophils), the presence of foreign or modified components, toxic substances.

Additionally, an immunological blood test is performed. If such an analysis shows too little IgG, it is highly likely that there is a malignant tumor process in the lymphatic system. A blood test for antibodies can show the presence of viruses in the body that can trigger the development of lymphoma.

As an option, in private clinics you can undergo a comprehensive molecular genetic study, which includes blood and urine tests, PRC analysis, testing for hormones, infections, viruses, etc., or even better, cancer screening of the body.

Next, in case of skin pathologies, a scraping is taken from the affected area, and if the lymph nodes are enlarged or damage to internal organs is suspected, a puncture biopsy is performed. The resulting material is sent for cytological analysis.

Instrumental diagnosis of T-cell lymphoma includes:

  • X-ray,
  • Computer and magnetic resonance imaging, which allows you to see the state of the body from the inside from different angles.
  • Ultrasound of the abdominal cavity for suspected mediastinal lymphoma.
  • Scintigraphy to detect metastases and bone lesions.
  • Computed axial tomography.

Depending on the type of T-cell lymphoma and its stage, the oncologist selects current methods for diagnosing the disease.

, , , , , , , , ,

Differential diagnosis

Differential diagnosis is an important point in establishing an accurate diagnosis, especially for cutaneous lymphomas, which in many ways resemble dermatological pathologies (for example, eczema, psoriasis, dermatitis), and pathologies accompanied by lymphadenopathy. The last point is very important, since enlarged lymph nodes can be both malignant (with lymphomas) and benign (with inflammatory processes in the body).

, , , , , , , , , ,

Treatment of T-cell lymphoma

Oncological pathologies have never been considered easy to treat, but T-cell lymphoma, despite the danger of the situation, is not a reason to give up. The treatment regimen and, to a greater extent, the outcome always depend on the timeliness of antitumor measures and the type of T-cell pathology.

The main methods of combating malignant lymphatic neoplasms are:

  • Systemic therapy with chemicals that stop tumor growth (chemotherapy).
  • Exposure of the tumor to ionizing radiation (electron beam therapy, which is the only method of physiotherapeutic treatment for malignant lymphomas).
  • Taking corticosteroids (anti-inflammatory therapy),
  • Taking drugs that activate antitumor processes in the body (biotherapy). The drugs are made directly from the patient's cellular structures.
  • Bone marrow transplant. In this case, intensive chemotherapy (myeloablative therapy) is first carried out, aimed at completely destroying cancer cells and suppressing the immune system to avoid transplant rejection, and then the patient is injected with healthy stem cells from a donor or their own, taken before chemotherapy.
  • Taking medications that stimulate the immune system and vitamins to boost immunity.
  • Surgical treatment. Surgical removal of the tumor is performed only for single lesions, mainly when the tumor process is diagnosed in the gastrointestinal tract. To avoid recurrence of the tumor after its removal, chemotherapy with several antitumor drugs simultaneously (polychemotherapy) is indicated.

Surgical treatment for T-cell lymphoma is not always justified. Firstly, in most cases, several lesions are detected, and the migration of pathological cells through the lymphatic system contributes to the fairly rapid spread of the disease to other organs (metastasis), which sometimes requires not one, but several operations. And if we take into account that cancer significantly weakens the body, then not every patient can endure even one, let alone several, surgical interventions.

The approach to treatment, of course, may be different depending on the location and extent of the process. Thus, for the treatment of mild forms of mycosis fungoides (erythematous), the use of corticosteroids and interferon preparations is indicated. Chemotherapy is not prescribed and radiation is not performed.

For other forms of T-cell cutaneous lymphomas, both systemic and local treatment may be prescribed. Local treatment of skin cancer T-pathologies can be carried out with the drug in the form of Valchlor gel, produced in the USA. Unfortunately, this drug is still not available in our country.

The active ingredient of the drug is mechlorethamine. Drugs containing this active substance (for example, Embiquin) are used for systemic therapy of T-cell forms of skin cancer. Also, as part of polychemotherapy, antitumor agents such as Vinblastine, Fludarabine, Dacarbazine, Chlorbutin, Adriamycin, Vincristine, Cyclophosphamide, etc. can be used.

For skin pathologies, antitumor antibiotics (for example, Rubomycin), corticosteroids (for example, Prednisolone, Triamcinolone) and phototherapy (usually PUVA therapy) are also used.

In the biotherapy of T-cell lymphomas, both patient cell preparations and monoclonal antibodies in the form of Rituxan, MabThera, Campas, Kampat, Avastin, and Bexar can be used.

Treatment of the consequences of high-dose myeloablative therapy is carried out using the drugs “Filstim”, “Zarsio”, “Neipomax”, etc.

Medicines can be prescribed either orally or intravenously. Most drugs are intended for drip administration. Treatment should be carried out under the strict supervision of an oncologist.

Radiation therapy for T-cell lymphoma is usually carried out over a course of 21-40 days. Irradiation is local in nature, without damaging other areas of the body. The time and dose of radiation is determined by the radiologist depending on the location and stage of the tumor process.

At the initial stages of the pathology, radiation therapy can be prescribed as an independent method of treatment, then it is used in combination with chemotherapy.

With smoldering forms of T-lymphoma, doctors are in no hurry to prescribe conservative treatment, taking a wait-and-see approach. The patient is regularly monitored by an oncologist, and in case of exacerbation, he will be prescribed effective therapy in accordance with the type of pathology.

Medicines for T-cell lymphoma

It is worth mentioning right away that the treatment of cancer of any etiology is a serious issue that should be dealt with by specialists. What types of drugs will be effective for a specific pathology, and what treatment regimen to use, is decided by a specialist doctor. Doctors categorically do not recommend prescribing treatment for yourself.

Today there are quite a lot of drugs for the treatment of tumor diseases. If we talk about their effectiveness, it is worth noting: timely initiation of therapy in most cases gives positive results. It is clear that even chemotherapy cannot treat advanced stages of cancer. In addition, each organism is individual, and what brings the desired relief to one does not always help save the life of another patient.

Let's look at just a few drugs that doctors use to treat T-cell lymphomas of various origins.

"Embiquin" is an alkylating cytostatic drug, the action of which is aimed at destroying the cellular structure of T-lymphocyte clones with gene mutations.

The drug is administered intravenously according to one of 2 schemes:

  • The impact method is designed for a 4-day course of chemotherapy. The patient is administered the drug once a day at a dosage defined as 0.1 mg per kilogram of weight. Sometimes the medicine is prescribed in full dosage once.
  • Fractional method. The medicine is administered 3 times a week. The usual dosage is 5-6 mg. Course – from 8 to 20 introductions.

Possible introduction into the pleural and abdominal cavities.

The drug is not prescribed for complicated course of the disease, severe anemia, certain blood diseases (leukopenia and thrombocytopenia). The question of the possibility of using the drug will also arise in the case of severe damage to the kidneys and liver, as well as in cardiovascular pathologies.

Common side effects include changes in blood characteristics, symptoms of anemia, gastrointestinal disturbances, and sometimes weakness and headaches. If the drug gets under the skin during injection, it can cause the formation of infiltration and tissue necrosis at the injection site. The severity of the main side effects during intensive therapy is much stronger than with fractional administration of the drug.

"Vinblastine" is an antineoplastic drug based on the vinca alkaloid. It has an antitumor effect due to the fact that it blocks cell division.

The drug can only be administered intravenously, trying to avoid getting under the skin. The dose is selected in accordance with the chosen chemotherapy regimen. The standard dosage is from 5.5 to 7.4 mg per 1 square meter of body surface (children's dose is from 3.75 to 5 mg per 1 sq.m.). The drug is prescribed once a week, sometimes once every 2 weeks.

There is another administration scheme, in which treatment begins with a minimum pediatric dose, gradually increasing it to 18.5 mg per 1 sq.m. bodies. For children, the initial dose will be 2.5 and the maximum 12.5 mg per 1 sq.m.

The course of treatment with the drug depends on the levels of leukocytes in the blood.

The drug is not used for severe suppression of bone marrow function, infectious pathologies, during pregnancy and breastfeeding. Do not use the medicine if you are hypersensitive to its components.

Common side effects: leukopenia and granulocytopenia. Violations from other organs and systems are observed much less frequently.

"Rubomycin" is a drug that belongs to the category of antitumor antibacterial agents, i.e. it simultaneously fights both cancer and bacterial cells.

The drug is also administered intravenously to avoid the appearance of infiltration and death of skin tissue. The medication is prescribed in a course of 5 days, during which the patient receives the drug at a dosage of 0.8 mg per 1 kg of weight. Repeat the course after 7-10 days. Now the drug is prescribed for a period of 3 to 5 days at a dosage of 0.5-1 mg per 1 kg of weight per day. Children's dose – from 1 to 1.5 mg per 1 kg of weight per day.

There are many cancer treatment regimens using the drug in combination with other anticancer drugs, in which the dosage and frequency of administration may differ.

Contraindications to the use of the drug are considered to be severe pathologies of the heart, blood vessels, liver and kidneys, suppression of bone marrow function, periods of pregnancy and lactation. In acute infectious pathologies there is a risk of developing various complications. Drinking alcohol is prohibited.

The most common side effects are granulocytopenia and trobrocytopenia (decreased concentrations of granulocytes and platelets in the blood).

“Campas” is a drug based on monoclonal antibodies that bind to lymphocytes and dissolve them, while bone marrow stem cells remain unaffected, which means the production of T-lymphocytes is not affected. Mature and maglinized lymphocytes die.

The drug is administered into the body by infusion, and the process of intravenous infusion is long and lasts at least 2 hours. To avoid allergic reactions and pain, analgesics and antihistamines are taken before the drip.

The medicine is administered over 3 days according to a scheme with increasing dosages: 3, 10 and 30 mg, while the body's reaction to the drug is constantly monitored. Then the medicine is administered every other day for 1-3 months. The dosage remains maximum - 30 mg per day.

If the drug is poorly tolerated and side effects occur, the dose is increased gradually only after the reaction to the drug returns to normal.

The drug is prohibited for use in patients with an acute stage of systemic infectious pathology, including cases of HIV infection, tumors of a non-lymphocytic nature, during pregnancy and during breastfeeding, and with hypersensitivity to the components. The drug is discontinued if toxic reactions are observed or further progression of the disease is observed.

There is not enough information about the effect of the drug on the children's body.

The most common side effects of the drug are: chills, fever, increased fatigue, decreased blood pressure, headaches. Many experience reactions from the gastrointestinal tract in the form of nausea, sometimes with vomiting, and diarrhea, changes in blood composition (decreased levels of granulocytes and platelets, anemia), hyperhidrosis, allergic reactions. Sepsis, herpes simplex, and pneumonia may also develop. Quite often, patients note a feeling of lack of air (dyspnea).

"Neipomax" is a drug that stimulates the production of leukocytes, the active substance is filgrastim.

Administration of large doses of chemotherapy before a bone marrow transplant often results in decreased white blood cell production, which must be corrected before the procedure begins. The same phenomenon can occur after conventional chemotherapy. Therefore, patients with neutropenia (insufficient production of neutrophil leukocytes) are prescribed drugs based on filgrastim.

The medicine is prescribed one day after chemotherapy at a dosage of 5 mcg per day. The medicine is administered subcutaneously. The course of treatment is no more than 14 days. Therapy is continued until the optimal number of neutrophils in the blood is achieved.

For myeloablative therapy, the drug is prescribed at a dosage of 10 mcg for 4 weeks. It is administered intravenously.

The medicine is not used for severe congenital neutropenia (Kastmann syndrome) and hypersensitivity to the components of the drug. Caution is exercised in case of sickle cell anemia.

Among the side effects of the drug are: dyspeptic symptoms, myalgia and arthralgia, an increase in the size of the liver and spleen, the appearance of an infiltrate in the lungs, bronchospasms and shortness of breath. Also sometimes there is a decrease in bone density, jumps in blood pressure, an increase in heart rate, swelling in the face, nosebleeds, and weakness. In some cases, changes in the composition of the blood are observed: a decrease in the level of platelets and an increase in the content of leukocytes (thrombocytopenia and leukocytosis). Protein and blood may be detected in the urine (protein and hematuria).

, , , [

For example, the effects of chemotherapy can be seen in the form of alopecia (active hair loss), weight gain due to increased appetite, heart rhythm disturbances and the development of heart failure. In some cases, a new diagnosis (usually a different type of blood cancer) is added to the existing diagnosis. For men, chemotherapy can lead to the inability to have children (infertility).

There are frequent cases of complications after radiation therapy. This may be a decrease in the functionality of the thyroid gland (as a rule, hypothyroidism develops), the development of radiation pneumonitis, characterized by scar formations in the tissues of the lungs, inflammation of the intestines or bladder (colitis and cystitis), insufficient production of saliva, radiation burns.

It turns out that we treat one thing and cripple another. This moment and the high cost of therapy for T-cell lymphoma and other oncological pathologies are pushing people to search for more and more new methods of treating this terrible disease.

On the Internet you can now find many posts about treating cancer with unconventional methods. Some people reject traditional treatment, citing the fact that it did not save the lives of friends, while others actively use it, achieving good results. We will not take one side or the other, but will just give some information about medications used outside the cancer hospital.

Many adherents of alternative cancer treatment (including many doctors!) agree that the cause of the development of malignant tumors is the acidic environment of the body, in which there is not enough oxygen for the normal functioning of cells. If you increase the pH level of the internal environment, cellular respiration improves and cell proliferation stops. It turns out that products that reduce the acidity of the body and improve the supply of oxygen to cells can stop the development of malignant processes.

A special role in this regard is given to a product that is present in virtually every kitchen - baking soda. As an alkali, it can reduce the acidity of any environment, including the internal environment of the body. It is recommended to use soda both orally (it alkalizes the gastrointestinal tract, entering the blood in some quantity) and in the form of injections, which allows alkali to be delivered to all corners of the body through the blood. However, it is recommended to extinguish the soda with boiling water first.

According to the method of the Italian doctor (!) Tulio Simoncini, soda should be taken twice a day, half an hour before meals (gradually increasing the dose from 1/5 to 2 tsp), diluting it with hot water and drinking a glass of liquid (water, milk). It is necessary that the soda solution comes into direct contact with cancer cells, so lotions, inhalations, injections, and douching are also used to treat various types of cancer.

One of the adherents of this method of treating cancer is our compatriot Professor Ivan Pavlovich Neumyvakin, who developed a system for treating various types of cancer using soda and hydrogen peroxide. In this case, not pure peroxide is used, but its solution (take from 1 to 10 drops of 3% hydrogen peroxide per 50 ml of water). Take the product three times a day, an hour before meals, increasing the number of peroxide drops by 1 every day (the first day - 1 drop, the second - two, etc.). After 10 days of treatment, take a 5-day break.

By the way, in order to reduce the acidity of the internal environment and block the path to cancer, scientists who adhere to alkalization of the body advise paying special attention to your diet, since our diet contains foods that can either increase or decrease pH. Alkalinizing foods include greens, fruits (except very sweet ones), dried apricots, almonds, milk and dairy products, berries, and vegetables. But meat, sausages and smoked meats, fish and seafood, eggs, baked goods, grape juice, jam, and preserves, on the contrary, increase the acidity of the body. By the way, lemon without sugar is considered an alkalizing product, despite its acidity, but if it is consumed with sugar, its effect will be exactly the opposite.

Of interest is a method of fighting cancer such as drinking beetroot juice, which also improves cellular respiration and is able to stop the growth and development of cancer cells. And if we also take into account the rich vitamin and mineral composition of the bright red root vegetable, then we can say with confidence that treatment with its help will help strengthen the immune, nervous and other systems of the body. And this will enable the body to independently fight various diseases, including cancer.

Taking beet juice goes well with traditional treatments and even reduces their negative effects. But there is also information about curing cancer with beetroot juice alone, which is much cheaper than a course of treatment in a cancer hospital.

For treatment, freshly squeezed juice, left in the refrigerator for at least 2 hours, is used, made from brightly colored table fruits. In order to obtain sufficient antitumor results, you need to drink 600 ml of juice during the day. You should drink the juice slightly warmed half an hour before meals. You can mix it with carrot juice.

Drink beetroot juice in small sips of 100 ml per dose daily. The course of treatment is long (1 year or more). To stabilize the condition, drink the juice in the amount of 1 glass per day.

Eating boiled beets is also useful, since with short heat treatment it actually does not lose its beneficial properties.

By the way, it was not in vain that the choice of traditional healers fell on beets. Its antitumor properties are also recognized by official medicine. One of the antitumor drugs has even been developed based on beets.

Many plants also have antitumor properties, and although herbal treatment for T-cell lymphoma and other types of cancer is not widespread, there are positive reviews about such treatment.

Even in official medicine, plants containing caryoclastic poisons that can kill pathogenic cells are recognized as antitumor agents. These poisons include alkaloids, lactones and cardiac glycosides found in many plants:

  • alkaloid-containing: brilliant and magnificent colchicum (tubers), celandine, barberry, rue, wormwood, yellow cornflower and some others,
  • lactone-containing: podophilus thyroid and Himalayan, eucommia, helenium, vernonia almond, gaillardia.
  • Containing cardiac glycosides: white peresupen, dioecious and chokeberry, mad cucumber, colocynth, avran officinalis, etc.

You need to understand that the above plants contain toxic substances, which means their dosage is strictly limited.

The fruits of Sophora japonica, radish seeds, clover, elderberry, and cinquefoil are also famous for their anti-inflammatory and antitumor effect. And extracts of ginseng, echinacea, and elleutherococcus can be used as immunomodulators for T-cell lymphoma. Such popular plants as nettle, calendula, plantain, dandelion, and string are not far behind them.

As for homeopathy, it does not attempt to cure completely aggressive malignant tumors, but some of its remedies are quite suitable as additional and preventative ones. So, if after traditional treatment of malignant lymphoma exacerbations are observed. A homeopathic doctor may prescribe long-term use of homeopathic medicines Barium carbonicum (5 granules under the tongue 2 times a day) and Konium (7 granules before bedtime).

Carcinosinum and Fitolyakka have proven themselves well as antitumor drugs, and Echinacea compositum, Mucosa compositum, Edas-308, Galium-Hel and other homeopathic medicines are used as immunostimulants.

Prevention

The issue of preventing the development of oncological pathologies with high mortality, which includes T-cell lymphoma, is quite complex. To be honest, scientists still do not know a clear answer to the question: how to prevent cancer from occurring.

If we consider those factors that increase the likelihood of developing cancer pathologies, we can understand that not all of them can be excluded from your life. People with a hereditary predisposition and immunodeficiencies will have to make a lot of effort to avoid the fate of relatives with cancer.

You need to understand that the risk of getting cancer is lower in those people who treat inflammatory pathologies (including dermatological ones) in a timely manner, preventing them from becoming chronic, do not work with carcinogens, live in an ecologically clean area, and are not exposed to radiation. Those. There is something to think about, because your life is at stake.

Treatment of conditions that can develop into cancer (precancerous conditions) also plays a huge role in cancer prevention. Herbalists have even developed a herbal mixture that prevents uncontrolled cell division. In its composition we find: leaves of nettle and plantain 5 g each, meadowsweet flowers and birch catkins 10 g each, licorice (roots) in the amount of 3 g.

The crushed raw materials are thoroughly mixed, 10 g of the composition is taken and 1 tbsp is brewed. boiling water, after which it is kept in a water bath for about 20 minutes. After straining, it turns out that the glass is incomplete. You need to top it up with boiled water.

], [

The worst prognosis is for angioimmunoblastic and T-lymphoblastic lymphomas if the process has spread to the bone marrow and other organs. With angioimmunoblastic T-cell lymphoma, patients usually die after 2-3 years, and only just over 30% live longer. Improvement in complicated lymphoblastic lymphoma can be observed only in a fifth of patients. Although if you start treating the disease at an early stage, the prognosis in most cases is quite favorable.

As for mycosis fungoides, everything depends on the form and stage of the pathology. The classic form is characterized by the best prognosis. Timely treatment in the absence of complications can give patients another 10 or more years of life. With complicated pathologies, life expectancy is reduced to 2-5 years. But with the Vidal-Brocca form, most patients die within one year.

T-cell lymphoma, like other cancers, feeds on our fear. Therefore, the prognosis for life largely depends on the patient’s mood. In medical practice, there have been cases of “miraculous” healing, when people who were on the edge of the grave, whom doctors could no longer help, recovered only because they believed in the possibility of victory over a fatal disease. No matter how the circumstances develop, you should never give up, because life is the greatest value for a person, and it is worth fighting for.

An oncohematologist is involved in identifying lymphoblastic lymphoma; if the above symptoms appear, you must consult a doctor to clarify the diagnosis. The doctor's actions include the following:

  • A survey about complaints, clarification of life history, illness and the presence of similar blood pathologies in close relatives.

  • Clinical examination.

  • Carrying out instrumental and laboratory diagnostic methods.

TO instrumental diagnostic methods lymphoblastic lymphoma include radiography, magnetic resonance and computed tomography, ultrasound and biopsy of the affected lymph nodes. It is the latter method that plays the most important role among all instrumental ones, as it allows one to determine the histological component of cancer.

In the photo: A variant of lymphoblastic B-cell lymphoma. We see cells that resemble their normal counterparts in the reactive germinal center. There is an admixture of split and uncleaved cells of medium and large size. The nuclei may be small, irregular and angular or oval with a few small nucleoli. The cytoplasm is sparse and there are numerous mitotic figures and apoptotic cells.

TO laboratory diagnostic methods Lymphoblastic lymphoma includes a complete blood count, blood biochemistry, enzyme immunoassay of a biopsy sample from a lymph node. Microscopically, a drop of blood is distinguished by an increased number of lymphocyte cells and their irregular structure. As a result of using the above methods, it is possible to accurately identify the disease and possibly determine the causes, which will allow you to select the most appropriate therapy.

T-cell lymphoma belongs to a group of non-Hodgkin cancer pathologies that affect the lymphatic system. This hematological disease mainly affects older men, but can occur in women. The disease is of epidernotropic origin and is characterized by aggressive development.

Classification of non-Hodgkin's diseases

The human lymphatic system is the main human defense against infectious diseases. The main assistants in the fight against viruses are lymphocytes.

These blood elements are divided into three types:

Non-Hodgkin's diseases occur due to modifications of these cells, which are capable of rapidly mutating and multiplying. Depending on the name of the leukocytes involved in the development of the disease, lymphomas are divided into NK-, B- and T-cell tumors.

Among B-cell non-Hodgkin neoplasms, the most common pathology is:

  • mantle cell lymphoma;
  • follicular tumor;
  • plasmacytoma;
  • neoplasm in the marginal zone;
  • small cell lymphoma.

NK cell tumors are formed from atypical NK leukocytes. Neoplasms caused by T cell mutations include:

These are diseases of different structure and structure, which are characterized by the proliferation of malignant cells in lymphoreticular tissues. The pathological process covers the bone marrow, lymph nodes, digestive system, liver and spleen. Non-Hodgkin's tumors are much more common than Hodgkin's disease.

Characteristics of T-cell lymphomas

Depending on the development of the disease, indolent and aggressive non-Hodgkin lymphomas are distinguished. False tissue disease is also extremely rare. This disease is similar to cancerous tumors, but is a neoplasm of a hematological nature.

Indolent pathologies are divided into several subtypes:


Indolent tumors are passive neoplasms of slow development. Aggressive lymphomas differ in the intensity of their growth. This disease includes:

  • Sézary syndrome;
  • aggressive and primary peripheral cutaneous T-cell lymphoma;
  • lymphoma is preliminary;
  • extranodal tumor;
  • adult leukemia.

Sometimes passive tumors can develop into lymphomas with an aggressive course of the disease. In other cases, T-cell pathology develops at an average rate.

Causes of pathology

The reasons for such changes in the lymphatic system have not been fully identified. Scientists believe that the main factor in the formation of malignant T-leukocyte tumors is the human leukemic T-cell virus type 1. Basically, there are several reasons that influence the development of T-lymphomas:

  • hereditary genetic predisposition;
  • influence on the body of chemicals, ultraviolet and radiation rays for a long time;
  • inflammatory processes in the body;
  • inherited immunodeficiency.

Elderly people are at risk. The combination of all these factors and constant stress, overwork of the body and poor nutrition can lead to changes in tissue cells. The result is the formation of T-cell lymphomas of the skin or cellular peripheral neoplasms.

Symptoms and diagnosis of the disease

According to the signs of development, cellular T-lymphoma is at 4 stages:

  1. The pathology affects only one area of ​​the lymph nodes.
  2. Pathology occurs only on one side of the diaphragm in the spirit nodes.
  3. Bilateral damage to the diaphragm.
  4. The modified cells grow and spread throughout the lymphatic system, affecting vital human organs.

The fourth stage disease can form metastases in the liver, stomach, kidneys and bone marrow. The disease often appears as a result of aggravated advanced pathology.

Symptoms of T cell modification can be different:

  • high degree of sweating;
  • sudden and prolonged weight loss;
  • digestive problems;
  • general weakness in the body, irritability and drowsiness;
  • changes in body temperature with deviations from the norm in one direction or the other.

With cutaneous lymphomas, nodules, spots, and rashes of various shapes appear in T cells.

If you detect any signs of changes in leukocytes, you should immediately consult a doctor. At the oncology center, an initial examination by an oncologist is carried out.

The next stage of diagnosing the disease is the complete morphology of the disease. A urine test and detection of antibodies in plasma cells are required.

A complete examination of a patient with suspected T-leukocyte modifications includes computer, magnetic resonance imaging and ultrasound.

The final prognosis of lymphoma is based on a comprehensive examination and depends on the type of lesion. Aggressive tumors require immediate treatment. The treatment program for these tumors mainly includes chemotherapy and radiation. A satisfactory result is positive remission after radiation therapy.

Isolated, according to the WHO classification, is represented by tumors from mature T cells and NK cells. T/NK cell lymphomas account for approximately 12% of all non-Hodgkin lymphomas.

Need for improvement classification of T-cell lymphomas This is explained by the fact that even when using the Kiel classification, which takes into account tumor immunophenotyping data, the reproducibility of the diagnosis is extremely low. For this reason, in the WHO classification, most T-cell lymphomas are combined into one group - peripheral T-cell lymphoma without further detail (unspecified). It should be recognized that the tumor pathology of peripheral T cells is so diverse that today immunological knowledge is far from sufficient for its classification generalization. The validity of this provision is fully demonstrated by the T-cell section of the WHO classification.

Mature T/NK cell tumors are characterized by a less favorable prognosis than B-cell tumors: the five-year survival rate does not exceed 30%. For this reason, establishing the T-lineage of lymphoma cells is of great clinical importance. The exception is anaplastic large cell lymphoma with a T-cell or null immunophenotype, for which the five-year survival rate exceeds 70%.

Peripheral T-cell tumors originate from mature or postthymic T cells. In the WHO classification (2001), NK cell tumors are combined with T cell tumors in one section, since NK cells have a number of immunophenotypic and functional properties in common with T cells. This position, however, cannot be considered indisputable. On the contrary, from an immunological point of view, such a union is more than conditional. At the same time, it should be recognized that due to the extreme rarity of NK-cell lymphomas (leukemia) and the lack of sufficient knowledge about their clinical course and prognosis, the classification of NK-cell tumors into a separate group does not currently have sufficient grounds.

Some properties of mature T- and NK-cell tumors are presented in the table.

Some features of T- and NK-cell lymphomas

According to WHO classification, according to clinical manifestations, mature T- and NK-cell tumors are divided into five main groups, highlighting variants within each group.

Using the example T cell lymphomas It is especially clear that the diagnosis of these forms of tumors cannot be only histological or even immunohistochemical. A significant proportion of the group of peripheral T-cell lymphomas are lymphoproliferations with a pronounced dominant or exclusively leukemic component, in which the diagnosis can be made based on the study of blood cells or bone marrow. In the group of leukemic/disseminated T/NK-cell leukemia (prolymphocytic leukemia, two variants of leukemia from large granular lymphocytes and endemic adult leukemia/lymphoma, which is less relevant for Russia), an accurate diagnosis is possible using immunological methods. Leukemic/disseminated lymphomas from T-helper (CD4) cells, as a rule, occur with high leukocytosis, while leukemic manifestations in T-cytotoxic/suppressor (CD8) and NK-cell peripheral lymphomas are not so pronounced due to the associated cytopenic (neutropenic) manifestations.

Peripheral T cell lymphomas, in which the diagnosis is mainly established by the results of histological examination of biopsied lymph nodes or other organs, can be divided into two types on the basis of immunological data.

Bulk tumors represent tumors of typical T cells expressing the T cell receptor (a/b or y/q) in association with the CD3 complex. Differential immunological diagnosis within this group is extremely complex (an exception may be T-cell lymphoma of the enteropathic type) and, as a rule, is based on confirmation of the T-cell nature of lymphomas that have specific histological manifestations. Features of tub cell homing may be associated with clinical manifestations in a rare group of hepatosplenic lymphomas.


Specific category tumors, included in the T-cell section, are essentially lymphomas of unspecified or unknown origin: T-cell receptor and CD3 expression are absent. These lymphomas require detailed immunological and molecular biological study.

It may be noted that diagnosis of specific variants of T-cell lymphomas is currently more morphological than immunological.

Diagnosis of T-cell lymphomas differs from the diagnosis of B-cell lymphomas in a number of features:
1) unlike B-cell lymphomas, specific immunophenotypic profiles do not have a direct association with most subtypes of T-cell lymphomas;
2) within each variant of peripheral T-cell lymphoma there is a spectrum of immunophenotypes;
3) there are no immunophenotypic markers of monoclonality. To confirm clonality in T-cell lymphomas, it is necessary to study the rearrangement of T-cell receptor genes using polymerase chain reaction (PCR). Membrane expression of TCR variable regions (eg, Vb) is studied using monoclonal antibodies;
4) in immunophenotypic diagnosis, an important role is played by establishing the aberrant immunophenotype of lymphoma T cells;
5) clinical, hematological and morphological signs play an important role in the diagnosis of variants of T-cell lymphomas.