Non-Hodgkin's lymphomas (lymphosarcoma) in adults

Types of lymphomas

There are two main groups of the disease – Hodgkin lymphoma and non-Hodgkin lymphoma. They develop from different types of cells, differ in their course and respond differently to therapy.

Hodgkin's lymphoma (HLH) is lymphogranulomatosis, malignant granuloma. Characterized by damage to B-lymphocytes. It has an aggressive course, but responds well to treatment. Source: I.I. Yakovtsova, V.D. Sadchikov, V.M. Sadchikova, O.V. Dolgaya, S.V. Danilyuk, A.E. Oleynik Hodgkin's lymphoma: evolution of clinical classifications // Bulletin of problems of biology and medicine, 2013, No. 3, v. 2 (103), pp. 62-69

Non-Hodgkin's lymphomas (NHL) affect not only B-, but also T-lymphocytes. A total of 30 subtypes have been identified. Most of them are less aggressive, but also more difficult to treat.

Classification of non-Hodgkin's lymphomas in accordance with the 4th edition of WHO 2008:

• B-lymphoblastic NHL;
• heavy chain disease;
• B-cell prolymphocytic leukemia;
• hairy cell leukemia;
• diffuse large B-cell NHL;
• Burkitt's leukemia;
• NHL from mantle zone cells;
• lymphoplasmacytic NHL;
• mediastinal diffuse large B-cell NHL;
• nodal marginal zone B-cell NHL;
• primary exudative NHL;
• plasma cell plasmacytoma/myeloma;
• splenic marginal zone NHL;
• follicular NHL;
• chronic lymphocytic leukemia;
• extranodal marginal zone B-cell NHL;
• T-lymphoblastic NHL;
• aggressive NK cell leukemia;
• anaplastic large cell NHL with primary skin lesions;
• anaplastic large cell NHL with primary systemic involvement;
• angioimmunoblastic T-cell NHL;
• hepatolienal T-cell NHL;
• Sézary's mycosis fungoides;
• unspecified peripheral T-cell NHL;
• T-cell panniculitis-like NHL of the subcutaneous tissue;
• T-cell leukemia;
• T-cell leukemia of large granular lymphocytes;
• T-cell prolymphocytic leukemia;
• extranodal NK/T-cell lymphoma of the nasal type;
• enteropathic T-cell NHL.

The most common lymphomas in clinical practice are: Hodgkin, follicular and diffuse large B-cell. Source: G.S. Tumyan Non-Hodgkin lymphoma // Clinical oncohematology, 2015, 8(4), pp.455-470

According to the nature of the lesion and pathogenesis, all lymphomas are divided into 3 main groups:

• aggressive – they are characterized by rapid development with pronounced symptoms;
• extranodal - the primary lesion is formed not in the lymph nodes, but immediately in the internal organs;
• indolent - distinguished by their slow and favorable course; may not require therapy; sometimes it is enough to see a doctor regularly.

For each type of lymphoma there is a special treatment regimen.

Lymphomas (lymphogranulomatosis and non-Hodgkin's lymphomas)

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IMPORTANT!

The information in this section cannot be used for self-diagnosis and self-treatment.
In case of pain or other exacerbation of the disease, diagnostic tests should be prescribed only by the attending physician. To make a diagnosis and properly prescribe treatment, you should contact your doctor. Lymphomas: causes, symptoms, diagnosis and treatment methods.

Definition

Lymphomas are a group of malignant tumor diseases of the lymphatic tissue, which under normal conditions is responsible for immunity. One of the first clinical symptoms of lymphoma is enlargement of lymph nodes in different locations.

Lymphomas, like other malignant tumors, have a tendency to metastasize, that is, the migration of tumor cells through the blood and lymph, as well as through contact into intact (healthy) tissues with subsequent reproduction in them.

Almost all types of lymphogranulomatosis, or Hodgkin's lymphoma, and about 65% of non-Hodgkin's lymphomas manifest as painless enlargement of the lymph nodes - 2 cm or more. However, the diagnosis of a particular type of lymphoma is established solely on the basis of a morphological examination of biopsy material taken from the tumor.

The incidence of Hodgkin's lymphoma in Russia is about 2 cases per 100 thousand population per year, and non-Hodgkin lymphoma is 5–7 cases per 100 thousand.

Causes of lymphomas

There are several possible causes for the development of lymphomas. It is important to understand that the basis for the development of any malignant disease, including lymphomas, is a dysfunction of cells.

The ability for uncontrolled tumor growth occurs when the natural process of cell division is disrupted: normally, the cell divides a limited number of times, matures, performs a certain function, and then ends its life.

Malignant cells are characterized by disruption of the processes of maturation and differentiation (the process during which the cell acquires specific characteristics and the ability to perform certain functions). Cells acquire new functions - they begin to produce proteins and toxins, and the ability to “escape immune surveillance” appears, as a result of which the process of natural cell death (apoptosis) changes.

Some viruses can directly affect the DNA of lymphocytes and contribute to their transformation into malignant cells.

For example, infection with Epstein–Barr virus (EBV) is an important risk factor for the development of lymphomas.

Lymphocytes are divided into two subtypes: T and B cells (T and B lymphocytes). Infection with human T-cell lymphotropic virus (HTLV-1) increases a patient's risk of developing certain types of T-cell lymphoma. The virus is transmitted sexually and through blood, as well as from mother to child through breast milk.

Viruses that weaken immunosurveillance are indirectly responsible for the development of certain types of lymphomas.

Thus, the human immunodeficiency virus (HIV) reduces the population of lymphocytes, while the control of mutant cells and herpes viruses type 8 and Epstein-Barr decreases with the formation of lymphomas.

Some infections can cause the development of lymphoma due to the constant activation of the immune system. Chronic infections produce a huge number of lymphocytes, which increases the likelihood of mutations during cell division and leads to the risk of lymphoma. Similar infections include Helicobacter pylori

(often found in chronic gastritis and peptic ulcers of the stomach and duodenum),
Chlamydophila psittaci
(risk of developing lymphoma in the tissues of the eye), hepatitis B and C viruses with prolonged infection.

Non-infectious risk factors for the development of lymphomas include:

• Exposure to chemicals. Numerous studies have shown that exposure to herbicides, insecticides, and benzene is associated with an increased risk of developing lymphomas.
• The presence of autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, etc.) is a significant risk factor for the development of lymphomas.
• Radiation exposure has a great influence on the rate of cell mutations and their malignancy.
• Genetic factors. Having a close relative with lymphoma greatly increases your risk.
• Congenital immune deficiency in certain diseases.
• Age. Some types of lymphoma develop in children and adolescents, while others, on the contrary, develop in people over 60 years of age.
• Breast implants can rarely lead to the development of a type of large cell lymphoma of the breast.

Classification of lymphomas
There are two main types of lymphomas: Hodgkin's lymphoma, or lymphogranulomatosis, and non-Hodgkin's lymphomas.

Hodgkin lymphoma is characterized by the presence of specific Reed-Berezovsky-Sternberg cells in the affected lymph nodes, spleen, liver or bone marrow.

Enlarged cervical lymph nodes in a patient with Hodgkin lymphoma

The group of non-Hodgkin's lymphomas includes:

• follicular lymphoma;
• marginal zone lymphoma;
• diffuse large B-cell lymphoma;
• mantle cell lymphoma;
• Burkitt's lymphoma;
• primary lymphoma of the central nervous system;
• nodal T-cell lymphomas;
• primary cutaneous lymphomas;
• non-Hodgkin's lymphomas in patients infected with HIV;
• non-Hodgkin's lymphoma in patients infected with hepatitis B and C viruses.

Symptoms of lymphomas
The earliest symptom is enlarged lymph nodes against the background of complete well-being. Enlarged lymph nodes are painless.

Various clinical manifestations depend on the location of the tumor: if the tumor grows in the lymph nodes of the chest, compression of the airways and the appearance of hoarseness, difficulty swallowing, difficulty breathing, and obsessive cough are possible.

When lymphoma is located in the mediastinum, compression syndrome of the superior vena cava appears: headaches, swelling and dilation of the veins of the face and neck occur.

In some cases, only general symptoms are present: loss of appetite, weight loss, increased body temperature, night sweats, weakness, and sometimes itchy skin and pain in enlarged lymph nodes.

Against the background of a decrease in immunity, infectious diseases can occur - the chickenpox virus is often activated, manifested by herpes zoster.

Cases of damage to the abdominal organs (manifested by an increase in the volume of the abdomen due to tumor growth and fluid accumulation, the appearance of pain, jaundice, nausea and vomiting) and bone marrow (pain in the bones, back, frequent fractures, pallor, increased bleeding) have been described.

Diagnosis of lymphomas

The diagnosis of a particular type of lymphoma is established solely on the basis of a morphological examination of biopsy material (a piece or a whole lymph node obtained during a minor operation) taken from the tumor (histological, cytogenetic and molecular genetic research methods). The doctor will prescribe additional tests to diagnose the stage of the disease and its complications. The list of studies depends on the presumed location of the tumor and may be changed.

• Clinical blood test.

Stages of lymphomas

Depending on the extent of the tumor, the stage of the pathological process is determined. In accordance with the international Ann Arbor classification, there are 4 stages of the disease:

• At stage 1, one group of lymph nodes may be involved in the lymphoma process.
• On the 2nd, the involvement of two or more groups on one side of the diaphragm is allowed.
• At stage 3, the lymph nodes on both sides of the diaphragm are affected.
• At the 4th stage, internal organs are involved in the process - the brain, heart, liver, spleen, stomach, intestines.

Each stage is additionally marked with titers A and B. Titer A indicates the absence of key symptoms - fever, severe night sweats and sudden weight loss. Titer B indicates that such manifestations exist.

Diagnostics

Typically, the diagnosis of lymphoma includes:

• Examination by a doctor and medical history.
• General laboratory tests, primarily blood tests.
• X-ray of the chest and other organs.
• Ultrasound of different types.
• , MRI.
• Radioisotope research methods.
• Biopsy of affected lymph nodes.

Depending on the situation, the diagnosis of lymphoma may be supplemented by specific studies at the discretion of the doctor.

Causes of lymphomas

A single causative factor for the development of the disease has not been identified. At the moment, scientists believe that the pathological process is triggered by a complex of reasons. These include:

• hereditary predisposition;
• regular contact with toxins and carcinogens;
• herpes virus, hepatitis, Helicobacter pylori and other infectious diseases;
• autoimmune and immunodeficiency conditions;
• recurring pneumonia;
• immunosuppressive therapy after kidney transplantation, stem cell transplantation, etc.;
• surgery to insert breast implants (presumably).

Weak immunity

As a rule, such lymphoma belongs to secondary oncology and develops against the background of metastasis. But primary pathology cannot be ruled out either. In this case, the most common reason for the development of the disease is a decrease in the body’s defenses. Of course, not a one-time event, but one that lasts for quite a long time. So, those at risk are those who:

• underwent organ transplant operations;
• has HIV;
• has Epstein-Barr virus;
• regularly exposed to radiation;
• stays in contact with carcinogens for a long time, including eating them;
• has severe heredity, including chromosomal mutations;
• lives in an area with poor ecology.

All this provokes abnormal cell division and the development of oncological processes in the brain.

Article on the topic

Immunity affected by cancer. Who is at risk for Hodgkin's lymphoma?

Symptoms

In addition to the main symptoms - fever, severe night sweats and sudden weight loss - the pathology is characterized by:

• weakness and increased fatigue;
• temperature increase;
• weight loss;
• enlarged lymph nodes (often, but not always; they are painless and do not shrink as a result of treatment with antibiotics, as with infectious diseases).

Sometimes itchy skin is possible. When a chest tumor is affected, difficulty breathing, shortness of breath and cough are possible. When the tumor is localized in the abdominal cavity, indigestion, nausea, vomiting, and bursting pain in the abdomen and lower back are observed. When the bone marrow is damaged, patients complain of pain in the bones. If the pathology involves facial and cranial structures, there are complaints of headaches and bursting pain in the face and neck.

How to recognize

The symptoms of the pathology are quite common, so they are often confused with other diseases of the central nervous system. Doctors identify the following symptoms in a patient with brain lymphoma:

• severe headaches;
• constant drowsiness - it doesn’t matter whether there are reasons for it or not;
• epilepsy attacks;
• emotional instability;
• neuropathy;
• speech and vision disorders;
• development of hallucinations;
• deterioration in coordination of movements;
• hand weakness.

In the last stages of the disease, the patient becomes inadequate and loses memory.

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More details

Diagnosis of lymphomas

The diagnosis is made based on the results of a microscopic examination of a biopsy - a sample of lymphoid tissue. Based on the results of a differential biopsy, the type of tumor is determined.

To identify tumors in different parts of the body, radiation diagnostic methods are used: radiography, scintigraphy, magnetic resonance, positron emission and computed tomography. If it is necessary to clarify some features of the pathological process, laboratory blood tests are prescribed - immunoenzyme typing using flow cytometry, molecular genetic and cytogenetic studies. Source: Wang HW, Balakrishna JP, Pittaluga S, Jaffe ES Diagnosis of Hodgkin lymphoma in the modern era // Br J Haematol. 2021 Jan;184(1):45-59. doi: 10.1111/bjh.15614. Epub 2021 Nov 8

3. Diagnosis of non-Hodgkin lymphoma

To diagnose lymphoma, a tissue biopsy is usually done.

If a patient has an enlarged lymph node that is not painful or associated with infection, a biopsy should be done.

During a biopsy, your doctor will take a piece of tissue from a lymph node or even remove the entire lymph node. And if the biopsy shows non-Hodgkin's lymphoma, further tests will be needed to determine the subtype of the disease and the stage of the disease. For this you may need:

• Blood analysis;
• Bone marrow biopsy;
• Chest X-ray;
• Computed tomography of the neck, chest, abdomen and pelvis;
• Spinal tap;
• X-ray of the small intestine;
• Endoscopy of the upper gastrointestinal tract;
• MRI for spinal or epidural lymphoma;
• Ultrasound of the testicle;
• Examination of the head and neck.

All of these tests will provide important information about the patient’s health and help determine the optimal treatment regimen for lymphoma.

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Treatment methods

The most common treatments for lymphomas are:

• radiation therapy;
• chemotherapy;
• radiotherapy;
• biological therapy;
• stem cell transplantation;
• surgical intervention.

They are used both as separate courses and in combination. The choice of treatment regimen depends on the type of tumor and the general condition of the patient.

Treatment tactics

Indolent lymphomas may not require treatment, but only observation by an oncohematologist. Therapy is indicated at the first signs of progression of the pathological process. For localized tumors, radiotherapeutic irradiation of the affected structures is sufficient. For generalized forms, chemotherapy is indicated.

In case of an aggressive course of the disease, one of the most effective treatment regimens is chemotherapy according to the CHOP protocol in combination with immunotherapeutic drugs from the group of monoclonal antibodies. For highly aggressive types of tumors, high-dose chemotherapy in combination with hematopoietic stem cell transplantation may be prescribed.

Treatment of certain types of lymphomas

The main method of treatment for Hodgkin lymphoma LGM is chemotherapy. Antitumor cytotoxins are prescribed in different dosage forms. Chemotherapy is cyclical in nature - drug courses alternate with periods of rehabilitation. Source: Sermer D, Pasqualucci L, Wendel HG, Melnick A, Younes A Emerging epigenetic-modulating therapies in lymphoma // Nat Rev Clin Oncol. 2019 Aug;16(8):494-507.doi: 10.1038/s41571-019-0190-8

Radiotherapy - high-power radiation - as an independent treatment for LM may be justified in some cases - when one, maximum two groups of lymph nodes are involved in the process. More often it is prescribed in combination with chemotherapy. In the later stages of the disease, the method is indicated as palliative treatment.

Biological drugs - monoclonal antibodies - are used in the treatment of lymphoma to activate the immune system. Radiation therapy is carried out regionally, in the area of ​​the lymph nodes involved in the oncological process.

In some cases, removal of the spleen is justified in the treatment of LGM. If enlarged lymph nodes are compressing neighboring organs, their excision may be indicated.

In case of relapses of the disease, as well as in order to destroy cancer cells that are not affected by standard doses of radiation and chemotherapy, high therapeutic doses are prescribed, which destroy bone marrow tissue along with the tumor. After such a course, organ transplantation is indicated. Your own or donor stem cells taken in advance form a new immune system.

A combination of radiation and chemotherapy also demonstrates good results in the treatment of NHL. In some cases, the innovative technique of lumbar puncture administration of antitumor antibodies is successfully used.

NHL with an aggressive course is difficult to treat. The most effective method to combat this disease is a bone marrow transplant.

How does lymphoma manifest: symptoms

The disease can develop asymptomatically; over time, the following nonspecific signs appear:

• temperature rise to 38-39 degrees and above;
• loss of appetite;
• weight loss;
• vomiting, nausea;
• general weakness, poor health;
• decreased performance;
• enlarged lymph nodes in several areas at once or only in one;
• itching in different places, including the affected area;
• pain in the head, chest, abdomen and other parts of the body.

If you palpate the lymph nodes, even pressing on them, the patient usually does not experience pain. At the same time, painful sensations from other parts of the body intensify when drinking alcohol. Another sign of lymphoma is the absence of pain in the nodes when taking antibacterial medications.

Lymphoma can also be associated with itching of the skin - this is observed in a third of cases. Itching appears all over the body or only in certain places. Moreover, such a symptom intensifies at night, and then may go away on its own.

The symptoms described do not necessarily indicate lymphoma. At the same time, there are a number of signs that can be attributed to specific manifestations of the disease:

• cough with chest pain;
• dyspnea;
• diarrhea, nausea;
• constipation;
• swelling in the area of ​​the affected lymph nodes.

Forecasts

The prognosis for patients with lymphoma depends on the stage of the process, the age and condition of the patient, as well as on the results of therapy. With Hodgkin's lymphoma, young patients in the early stages usually make a full recovery. In 8 out of 10, stable remission is achieved. The five-year survival rate for stage 1 is 95%, for stage 4 – 65%.

For NHL of the spleen, lymph nodes and mucous membranes, the average five-year survival rate is about 70%. For NHL of the gastrointestinal tract, salivary glands, and eye orbits, the average five-year survival rate is about 60%. The prognosis for aggressive NHL of the mammary glands, ovaries, central nervous system and bones is less favorable - the five-year survival rate is on average less than 30%.

Clinical recommendations after lymphoma treatment and prevention

Avoid risk factors - intoxication and contact with carcinogens. Get vaccinated, and if you get an infectious disease, do not self-medicate.

At the first sign of lymphoma, consult your doctor. Get annual preventive medical examinations, eat right, and try to lead a healthy lifestyle.

Sources:

• I.I. Yakovtsova, V.D. Sadchikov, V.M. Sadchikova, O.V. Dolgaya, S.V. Danilyuk, A.E. Oleinik. Hodgkin's lymphoma: evolution of clinical classifications // Bulletin of problems in biology and medicine, 2013, No. 3, vol. 2 (103), pp. 62-69.
• G.S. Tumyan. Non-Hodgkin's lymphomas // Clinical oncohematology, 2015, 8(4), pp.455-470.
• Wang HW, Balakrishna JP, Pittaluga S, Jaffe E. Diagnosis of Hodgkin lymphoma in the modern era // Br J Haematol. 2019 Jan;184(1):45-59. doi: 10.1111/bjh.15614. Epub 2021 Nov 8.
• Sermer D, Pasqualucci L, Wendel HG, Melnick A, Younes A. Emerging epigenetic-modulating therapies in lymphoma // Nat Rev Clin Oncol. 2021 Aug;16(8):494-507.doi: 10.1038/s41571-019-0190-8.

The information in this article is provided for reference purposes and does not replace advice from a qualified professional. Don't self-medicate! At the first signs of illness, you should consult a doctor.