C85.9 Non-Hodgkin's lymphoma, type unspecified Non-Hodgkin's lymphoma Non-Hodgkin's lymphoma ICD code 10

For all morphological variants of non-Hodgkin's lymphomas, there is an equally frequent lesion of both the lymph nodes in general and their individual groups, the Waldeyer lymphoid ring and the gastrointestinal tract. More frequent primary involvement of retroperitoneal lymph nodes and abdominal cavity, bones and soft tissues is observed with lymphoblastic, spleen - with prolymphocytic variants. The pathological process, regardless of the morphological variant of the disease, in most cases first spreads not to the zones adjacent to the lymph nodes. The defeat of adjacent groups of lymph nodes often occurs in the lymphoblastic variant.
Early extranodal metastasis, bone marrow metastasis, involvement of the liver and spleen in the pathological process are somewhat more common in the prolymphocytic variant, and bone marrow damage and leukemization are more common in the presence of cells with a rounded and split nucleus. At the same time, with blast variants, the involvement bone marrow and the increase in the size of the lymph nodes occur earlier.
The greatest differences between morphological variants are noted when assessing survival. The five-year survival rate for the prolymphocytic variant of small cells with split and round nuclei is 70 and 53%, respectively. In the prolymphocytic-lymphoblastic variant of large cells with a split nucleus, the survival rates approach those in blast variants and are 14-21 months.
Survival rates in stages I-II of non-Hodgkin's lymphomas with a high degree of malignancy in the primary lesion of the gastrointestinal tract significantly exceed those observed in the general group of patients with these variants.
Primary non-Hodgkin's lymphoma of the spleen is a rare localization (less than 1%), while its involvement in the pathological process is often (40-50%) found in lymphosarcomas. Somewhat more often, the primary lesion of the spleen is found in the prolymphocytic variant. More often, with spleen lymphoma, bone marrow is involved in the pathological process. However, in the lymphoblastic variant, metastases from the spleen are more often localized in the abdominal lymph nodes.
The most common lung involvement is found in low-grade non-Hodgkin's lymphomas. The prognosis for this primary localization is also determined by the morphological variant. The defeat of the nervous system ascertain, as a rule, with blast variants of non-Hodgkin's lymphomas.
The nodular type of non-Hodgkin's lymphomas within any histological type is characterized by a more favorable course of the disease. In the lymphocytic variant, despite the rapid generalization of the process, a relatively benign course is also noted.
The clinical and hematological picture in certain morphological variants of diffuse lymphosarcomas has its own characteristics. Thus, the lymphocytic variant is characterized by a rather early generalization of the process. Unlike chronic lymphocytic leukemia, it is often possible to trace the sequence of involvement and the pathological process of various groups of lymph nodes; histological examination of the bone marrow reveals a nodular or nodular-diffuse type of lesion (and unlike the diffuse nature of infiltration in chronic lymphocytic leukemia).
Generalization of the process, on average, occurs after 3-24 months. Bone marrow damage can also be detected with a normal hemogram (in 47% of patients it is not changed at the time of diagnosis), in some patients lymphocytopenia is detected. Despite the early generalization and involvement of the bone marrow in the process, the prognosis of the disease in this variant is relatively favorable (up to 75% of patients live for more than 5 years).
The T-cell variant of lymphosarcoma is distinguished by clinical and hematological peculiarities: splenomegaly, generalized enlargement of lymph nodes, infiltrates in the lungs, skin lesions. The primary focus is a T-dependent paracortical region of the lymph nodes. There is a high lymphocytosis in the blood, the nuclei of most of the lymphocytes are twisted. Average duration life with this rare variant is short - 10 months.
With a rare lymphoplasmacytic cytological variant clinical syndromes the course of the disease is determined by the localization of the tumor, the degree of prevalence of the process, often - the amount of IgM in the blood serum.
The prolymphocytic variant is found in 45-51% of all cases of lymphosarcoma. With it, an increase in the occipital, parotid, popliteal, and lymph nodes is often detected. Despite the uneven generalization and frequent leukemization (in 25-45%) of the process, with this option, the five-year survival rate of patients is 63-70%. In the prolymphocyte-lymphoblastic subvariant, the prognosis is less favorable.
The lymphoblastic variant, rather heterogeneous in its morphological (twisted, non-twisted nucleus, macro-, microforms) and immunological (T- and B-phenotype) characteristics, is most common in children. Are amazed The lymph nodes different localization. The disease is distinguished by the rapid growth of tumors and the involvement of new anatomical zones in the process. More often than in other lymphosarcomas, the initial cytopenia, T-cell phenotype of lymphocytes, is found in the hemogram.
Burkitt's lymphoma of B-cell origin is attributed to the lymphoblastic type of lymphosarcoma. Its classic variant is manifested mainly by bone lesions (especially mandible), kidneys, ovaries, lymph nodes of the retroperitoneal regions, lungs, parotid salivary glands. The bone marrow is rarely involved in the process. With localized forms, the prognosis is favorable with long-term remissions up to a complete cure. The most common type of T-lymphoblastic lymphoma is "prothymocyte". In the vast majority of cases, the mediastinum is affected, metastases are detected in the central nervous system, lungs; in 50% of cases - leukemization. The disease is more often detected in boys of the first 5 years of life and adolescents 13-16 years old.
Immunoblastic lymphosarcoma (predominantly B-cell phenotype) can develop as a primary tumor of the gastrointestinal tract, lymph nodes, Waldeyer's rings, and cytopenia is often detected, leukemization - in rare cases. The disease progresses rapidly, the five-year survival rate of patients is 21-32%, however, removal of a solitary tumor can contribute to many years of remission and even cure. Immunoblastic lymphosarcoma as a secondary process is described in multiple myeloma, Waldenström's macroglobulinemia, and other lymphoproliferative diseases.
Mycosis fungoides is a malignant lymphoid tumor that always occurs primarily in the upper layers of the dermis, consisting of polymorphic T-helpers. The first manifestation of the disease may be nonspecific inflammation. The diagnosis is verified according to histological, cytochemical studies (lymphoid cells give positive reaction for acid phosphatase, beta-glucuronidase and acid nonspecific esterase). There is a point of view that the early, chronic phase of the disease may be reactive, and the "lymphoblastic" represents a true malignant transformation. Cesari's syndrome, characterized by the appearance in the hemogram of lymphoid cells with a brain-shaped nucleus, is considered as the leukemic phase of mycosis fungoides.
The histiocytic variant of malignant non-Hodgkin's lymphomas is rare. Clinical picture its varied. Metastases can be found in many organs. Leukemization and bone marrow involvement are rare, with cytopenia often present.
The nosological affiliation of the identified new forms remains debatable. So, Lennert's lymphoma, originally described as an unusual variant of lymphogranulomatosis with a high content of epithelioid cells, is proposed to be considered an independent form. The absence of typical Berezovsky-Sternberg cells, fibrosis, a high content of immunoblasts, plasma cells, transitions to lymphosarcoma served as the basis for distinguishing this disease from lymphogranulomatosis and isolating it under the name "Lennert's lymphoma" (malignant lymphoma with a high content of epithelioid histiocytes, lymphoepithelial lymphoma, epithelioid cell lymphoma ). A feature of the clinical manifestations of Lennert's lymphoma is the frequent involvement of the palatine tonsils, lymph nodes, elderly age patients, the presence of polyclonal gammopathy and allergic skin rashes in history.
It is proposed to refer to non-Hodgkin's lymphomas also described in last years angioimmunoblastic lymphadenopathy with dysproteinemia (lymphogranulomatosis X). Clinically, the disease is manifested by fever, weight loss, skin rashes, generalized lymphadenopathy, often in combination with hepato- and splenomegaly, persistent hyperglobulinemia, sometimes signs of hemolysis. Histologically, a triad is characteristic: proliferation of small vessels, proliferation of immunoblasts, deposits of PAS-positive amorphous masses in the walls of blood vessels. The number of eosinophils and histiocytes fluctuates, but sometimes the number of the latter is markedly increased. Perhaps the presence of giant cells, small foci of necrosis. A number of researchers regard the changes described above not as malignant lymphoma, but as reactive, associated with disorders in the B-lymphocyte system.
Lymphocytes can be localized in various organs and tissues (spleen, lymph nodes, stomach, lungs, skin, etc.). The disease progresses slowly. For a long time, the spleen is slightly enlarged, the lymph nodes are of normal size or slightly enlarged. In the blood, the number of leukocytes is normal or close to normal, with a predominance or normal content of mature lymphocytes. The level of platelets is within the normal range, their number may decrease to 1 * 109 / l-1.4 * 109 / l in some patients after 7-10 years. More often, only a slight tendency to a decrease in the level of hemoglobin and the number of erythrocytes is revealed, reticulocytes fluctuate within 1.5-2%. Bone marrow biopsy reveals individual proliferates consisting of mature lymphocytes; histological studies an enlarged lymph node, other affected organs help to verify the diagnosis. Malignancy of a lymphocytoma with transformation into lymphosarcoma or chronic lymphocytic leukemia is not mandatory, and if it does occur, it is often after many months or years.

Diagnostic criteria

The diagnosis of HL should be made according to the WHO classification based on lymph node biopsy followed by histological examination and, if necessary, IHC.


Complaints and anamnesis: complaint of an increase in a certain group of peripheral lymph nodes, fever, sweating, pruritus, weight loss. Detailed history taking special attention to the presence of symptoms of intoxication, "alcoholic" pain (the appearance of pain in the affected areas after taking even a small amount of alcohol) and the growth rate of the lymph nodes.


Physical examination: thorough palpation examination of all groups of peripheral lymph nodes (submandibular, cervical-supraclavicular, subclavian, axillary, iliac, inguinal, femoral, ulnar, occipital), liver, spleen.


Laboratory research

Clinical Analysis blood, including the content of erythrocytes, hemoglobin, platelets, ESR.

Biochemical analysis blood (including the study of creatinine, urea, bilirubin, total protein, transaminases, LDH, alkaline phosphatase).

Norm Pathology
Total protein 66-87 g/l Hypoproteinenia 45-50 g/l
Urea 2.3-8.3 mmol/l Above 8.3 mmol/l
Creatinine 45-115 mmol/l Above 115 mmol/l
ALAT up to 0.68 (up to 0.52) mg/l Above 0.68 mg/l
ASAT up to 0.62 (up to 0.52) mkat/l Above 0.62mkat/l
Alkaline phosphatase 30 to 120 U/L Above 120U/L
LDH from 0 to 248 U/l Above 248 U/l
Bilirubin up to 22 µmol/l Above 22 µmol/l


Blood on RW.

Blood for HIV.

Histological examination.

Immunophenotypic study.

Instrumental Research

1. Radiography of organs chest.

3. Ultrasound:
- all groups of peripheral lymph nodes, including cervical, supraclavicular, axillary, inguinal, femoral;
- abdominal cavity.

4. Computed tomography of the chest and abdomen.

5. Computed tomography with contrast.

6. X-ray of bones if the patient complains of pain, as well as when changes are detected on scintigrams.

7. Radioisotope diagnostics to detect subclinical lesions of the skeletal system.

8. Scanning of the lymph nodes with gallium citrate, to detect early relapses.

9. Biopsy of the bone marrow (puncture or trepanobiopsy).

10. Magnetic resonance imaging.

11. Positron emission tomography.


Indications for expert advice:

1. Examination by an ENT doctor (palatine tonsils, nasopharynx) to exclude damage to the nasopharynx.

2. Examination by a radiologist to resolve the issue of radiation therapy.

3. Examination by a cardiologist in the presence of a history of cardiac diseases.

4. Examination by an endocrinologist in the presence of a history of diabetes mellitus.

5. Examination of the surgeon at emergency conditions and, if necessary, exploratory laparotomy and splenectomy.

6. Neurosurgeon and neuropathologist with lesions of the brain and spinal cord.

7. Optometrist with damage to the orbit of the eyes.

8. Obstetrician-gynecologist.

9. Angiosurgeon according to indications.


List of basic and additional diagnostic measures


The required amount of research before planned hospitalization:

1. Clinical blood test, including the content of red blood cells, hemoglobin, platelets, leukocyte formula, ESR.

2. Biochemical blood test, including the study of total protein, creatinine, urea, bilirubin, transaminases, LDH, alkaline phosphatase.

3. Determination of blood group and Rh factor.

4. Coagulogram.

7. X-ray of the chest.

8. Morphological examination of the bone marrow.

9. Trepanobiopsy of the iliac wing.

10. Ultrasound of the abdominal organs with a study of the liver, spleen.

11. EFGDS.

12. Histological examination.

13. Immunophenotypic study.

14. Buck. blood culture for hemoculture.


List of main diagnostic measures:

1. Excisional biopsy. For research, the earliest of the appeared lymph nodes is taken, which is removed completely. When removed, the assembly must not be mechanically damaged. It is undesirable to use inguinal lymph nodes for histological examination if there are other groups of lymph nodes involved in the process. Needle biopsy for initial diagnosis is insufficient. In difficult cases differential diagnosis with other lymphoproliferative diseases or solid tumors, an immunohistochemical study is necessary.

2. Transthoracic biopsy of mediastinal lymph nodes, enovideotoracoscopic biopsy of mediastinal lymph nodes.

3. Laparotomy verification of retroperitoneal lymph nodes.

4. Ultrasound:
- all groups of peripheral lymph nodes, including cervical, supra- and subclavian, axillary, inguinal, femoral, retroperitoneal;
- abdominal cavity.

B-cell lymphoma is a malignant neoplasm, the development of which is accompanied by the spread of cancer cells to other organs and systems in the body. To name exactly what causes can cause such a pathology, today it is impossible.

One thing is certain: the earlier B-cell lymphoma is diagnosed, the greater the chance of a full recovery. According to many researchers, the development of neoplasms can be influenced by toxic and carcinogenic substances when they affect the human body.

General characteristics and causes of pathology

The International Classification of Diseases 10th revision (ICD 10) assigns the code C85.1 - B-cell lymphoma, unspecified.

According to numerous studies in some countries, it is cellular (large cell) lymphoma that is a disease that has become epidemic. The main reason for this phenomenon is the increase in cases of acquired and congenital immunodeficiency.

Due to the rapid progression of the symptoms of the disease, the rapid development of insufficiency of any internal organ especially if therapy is started late. Thanks to drugs - cytostatics, which appeared on the market relatively recently, you can significantly increase the chances of a favorable prognosis.

Despite insufficient study of the reasons contributing to the occurrence of such malignant neoplasm, like lymphoma, factors predisposing to pathology can be distinguished:

  • development in the body of acquired immunodeficiency syndrome (AIDS, HIV);
  • presence viral infection that provokes the development of a disease such as hepatitis;
  • development of autoimmune thyroiditis;
  • the development of a genetic pathology, for example, Klinefelter's syndrome;
  • exposure to the body of a chemical aggressive substance or radiation;
  • unfavorable environmental conditions in which a person lives;
  • development of congenital immune pathology;
  • age factor;
  • development of rheumatoid arthritis;
  • excess weight;
  • radiotherapy or chemotherapy to eliminate cancer.

Neoplasm classification

Benign B-cell lymphoma is divided into certain types according to the European-American classification developed by the World Health Organization:


Symptoms and signs that characterize B-cell lymphoma

B-cell lymphoma has similar symptoms to some types malignant tumors. Common non-specific symptoms include:

  • sudden and unreasonable weight loss;
  • slight increase in overall temperature;
  • general malaise;
  • lymph nodes begin to increase in groups;
  • fatigue, even with minor loads;
  • increased sweating, especially at night;
  • development of anemia, thrombocytopenia, which causes symptoms such as pallor skin and increased bleeding.

If the bone apparatus or internal organs are involved in the lesion, pain occurs in the corresponding area and other characteristic symptoms of B-cell lymphoma (ICB code 10 - C85.1):

  • with lung damage - a feeling of lack of air and cough;
  • with intestinal damage - impaired digestion, vomiting;
  • with damage - frequent dizziness, headaches, impaired visual system.

How is β-cell lymphoma diagnosed?

To make a correct diagnosis, immediately after a person goes to the hospital with characteristic symptoms the doctor prescribes certain instrumental and laboratory tests:

  1. Ultrasound procedure, which is carried out to determine the state of the affected lymph nodes and internal organs.
  2. X-ray examination, which is assigned to reveal pathological changes in bone tissue, thoracic and abdominal organs.
  3. Carrying out a bone marrow puncture- an invasive study is necessary to study the taken sample with subsequent genetic, immunological and cytological analysis. In this case, you can determine the type of neoplasm and further prognosis.
  4. Magnetic resonance imaging and CT scan help determine the degree of damage to a particular internal organ, as well as the stage of development of B-cell lymphoma.
  5. Performing a lumbar puncture prescribed in order to determine the degree of spread of pathological lymphoma cells in the central nervous system.
  6. A biopsy is a diagnostic method, in the process of which it is possible to determine the type of lymphoma and the stage of its development.

Treatment and prognosis

To achieve a complete recovery or a stable remission, it is necessary complex treatment B-cell lymphoma using all possible methods. First of all, it is necessary to take immunomodulators, antibiotic, antiviral and antitumor agents.

Chemotherapeutic treatment of B-cell lymphoma consists in the use of potent medicines, which pathologically affect cancer cells. It is advisable to use Doxorubicin, Vinblastine, Bleomycin for two courses of chemotherapy.

Another method can be used intensive care B-cell lymphoma, such as X-ray radiation, which is aimed at combating cancer cells, as well as preventing their spread to nearby tissues. Such treatment is effective only at the first stage of the development of pathology.

The prognosis of B-cell lymphoma depends on how timely the neoplasm was diagnosed, as well as on the correctness of the treatment performed. The percentage of survival is determined taking into account the gender and biological age of the patient, the state of immunity, the type of neoplasm.

B-cell lymphoma (ICD code 10 - C85.1) is curable, but only with timely initiation of therapy. The positive attitude of the patient is 50% of success in treatment.

For all morphological variants of non-Hodgkin's lymphomas, there is an equally frequent lesion of both the lymph nodes in general and their individual groups, the Waldeyer lymphoid ring and the gastrointestinal tract. A more frequent primary lesion of the retroperitoneal lymph nodes and the abdominal cavity, bones and soft tissues is observed with lymphoblastic, spleen - with prolymphocytic variants. The pathological process, regardless of the morphological variant of the disease, in most cases first spreads not to the zones adjacent to the lymph nodes. The defeat of adjacent groups of lymph nodes often occurs in the lymphoblastic variant.
Early extranodal metastasis, bone marrow metastasis, involvement of the liver and spleen in the pathological process are somewhat more common in the prolymphocytic variant, and bone marrow damage and leukemization are more common in the presence of cells with a rounded and split nucleus. At the same time, in blast variants, the involvement of the bone marrow and the increase in the size of the lymph nodes occur earlier.
The greatest differences between morphological variants are noted when assessing survival. The five-year survival rate for the prolymphocytic variant of small cells with split and round nuclei is 70 and 53%, respectively. In the prolymphocytic-lymphoblastic variant of large cells with a split nucleus, the survival rates approach those in blast variants and are 14-21 months.
Survival rates in stages I-II of non-Hodgkin's lymphomas with a high degree of malignancy in the primary lesion of the gastrointestinal tract significantly exceed those observed in the general group of patients with these variants.
Primary non-Hodgkin's lymphoma of the spleen is a rare localization (less than 1%), while its involvement in the pathological process is often (40-50%) found in lymphosarcomas. Somewhat more often, the primary lesion of the spleen is found in the prolymphocytic variant. More often, with spleen lymphoma, bone marrow is involved in the pathological process. However, in the lymphoblastic variant, metastases from the spleen are more often localized in the abdominal lymph nodes.
The most common lung involvement is found in low-grade non-Hodgkin's lymphomas. The prognosis for this primary localization is also determined by the morphological variant. The defeat of the nervous system ascertain, as a rule, with blast variants of non-Hodgkin's lymphomas.
The nodular type of non-Hodgkin's lymphomas within any histological type is characterized by a more favorable course of the disease. In the lymphocytic variant, despite the rapid generalization of the process, a relatively benign course is also noted.
The clinical and hematological picture in certain morphological variants of diffuse lymphosarcomas has its own characteristics. Thus, the lymphocytic variant is characterized by a rather early generalization of the process. Unlike chronic lymphocytic leukemia, it is often possible to trace the sequence of involvement and the pathological process of various groups of lymph nodes; histological examination of the bone marrow reveals a nodular or nodular-diffuse type of lesion (and unlike the diffuse nature of infiltration in chronic lymphocytic leukemia).
Generalization of the process, on average, occurs after 3-24 months. Bone marrow damage can also be detected with a normal hemogram (in 47% of patients it is not changed at the time of diagnosis), in some patients lymphocytopenia is detected. Despite the early generalization and involvement of the bone marrow in the process, the prognosis of the disease in this variant is relatively favorable (up to 75% of patients live for more than 5 years).
The T-cell variant of lymphosarcoma is distinguished by clinical and hematological peculiarities: splenomegaly, generalized enlargement of lymph nodes, infiltrates in the lungs, skin lesions. The primary focus is a T-dependent paracortical region of the lymph nodes. There is a high lymphocytosis in the blood, the nuclei of most of the lymphocytes are twisted. The average life expectancy in this rare variant is short - 10 months.
With a rare lymphoplasmacytic cytological variant, the clinical syndromes of the course of the disease are determined by the localization of the tumor, the degree of prevalence of the process, and often by the amount of IgM in the blood serum.
The prolymphocytic variant is found in 45-51% of all cases of lymphosarcoma. With it, an increase in the occipital, parotid, popliteal, and lymph nodes is often detected. Despite the uneven generalization and frequent leukemization (in 25-45%) of the process, with this option, the five-year survival rate of patients is 63-70%. In the prolymphocyte-lymphoblastic subvariant, the prognosis is less favorable.
The lymphoblastic variant, rather heterogeneous in its morphological (twisted, non-twisted nucleus, macro-, microforms) and immunological (T- and B-phenotype) characteristics, is most common in children. Lymph nodes of various localization are affected. The disease is distinguished by the rapid growth of tumors and the involvement of new anatomical zones in the process. More often than in other lymphosarcomas, the initial cytopenia, T-cell phenotype of lymphocytes, is found in the hemogram.
Burkitt's lymphoma of B-cell origin is attributed to the lymphoblastic type of lymphosarcoma. Its classic variant is manifested mainly by damage to the bones (especially the lower jaw), kidneys, ovaries, lymph nodes of the retroperitoneal regions, lungs, parotid salivary glands. The bone marrow is rarely involved in the process. With localized forms, the prognosis is favorable with long-term remissions up to a complete cure. The most common type of T-lymphoblastic lymphoma is "prothymocyte". In the vast majority of cases, the mediastinum is affected, metastases are detected in the central nervous system, lungs; in 50% of cases - leukemization. The disease is more often detected in boys of the first 5 years of life and adolescents 13-16 years old.
Immunoblastic lymphosarcoma (predominantly B-cell phenotype) can develop as a primary tumor of the gastrointestinal tract, lymph nodes, Waldeyer's rings, and cytopenia is often detected, leukemization - in rare cases. The disease progresses rapidly, the five-year survival rate of patients is 21-32%, however, removal of a solitary tumor can contribute to many years of remission and even cure. Immunoblastic lymphosarcoma as a secondary process is described in multiple myeloma, Waldenström's macroglobulinemia, and other lymphoproliferative diseases.
Mycosis fungoides is a malignant lymphoid tumor that always occurs primarily in the upper layers of the dermis, consisting of polymorphic T-helpers. The first manifestation of the disease may be nonspecific inflammation. The diagnosis is verified according to histological, cytochemical studies (lymphoid cells give a positive reaction to acid phosphatase, beta-glucuronidase and nonspecific acid esterase). There is a point of view that the early, chronic phase of the disease may be reactive, and the "lymphoblastic" represents a true malignant transformation. Cesari's syndrome, characterized by the appearance in the hemogram of lymphoid cells with a brain-shaped nucleus, is considered as the leukemic phase of mycosis fungoides.
The histiocytic variant of malignant non-Hodgkin's lymphomas is rare. Its clinical picture is varied. Metastases can be found in many organs. Leukemization and bone marrow involvement are rare, with cytopenia often present.
The nosological affiliation of the identified new forms remains debatable. So, Lennert's lymphoma, originally described as an unusual variant of lymphogranulomatosis with a high content of epithelioid cells, is proposed to be considered an independent form. The absence of typical Berezovsky-Sternberg cells, fibrosis, a high content of immunoblasts, plasma cells, transitions to lymphosarcoma served as the basis for distinguishing this disease from lymphogranulomatosis and isolating it under the name "Lennert's lymphoma" (malignant lymphoma with a high content of epithelioid histiocytes, lymphoepithelial lymphoma, epithelioid cell lymphoma ). A feature of the clinical manifestations of Lennert's lymphoma is the frequent defeat of the palatine tonsils of the lymph nodes, the elderly age of patients, the presence of polyclonal gammopathy and allergic skin rashes in history.
Angioimmunoblastic lymphadenopathy with dysproteinemia (lymphogranulomatosis X), also described in recent years, is also proposed to be referred to non-Hodgkin's lymphomas. Clinically, the disease is manifested by fever, weight loss, skin rashes, generalized lymphadenopathy, often in combination with hepato- and splenomegaly, persistent hyperglobulinemia, and sometimes signs of hemolysis. Histologically, a triad is characteristic: proliferation of small vessels, proliferation of immunoblasts, deposits of PAS-positive amorphous masses in the walls of blood vessels. The number of eosinophils and histiocytes fluctuates, but sometimes the number of the latter is markedly increased. Perhaps the presence of giant cells, small foci of necrosis. A number of researchers regard the changes described above not as malignant lymphoma, but as reactive, associated with disorders in the B-lymphocyte system.
Lymphocytes can be localized in various organs and tissues (spleen, lymph nodes, stomach, lungs, skin, etc.). The disease progresses slowly. For a long time, the spleen is slightly enlarged, the lymph nodes are of normal size or slightly enlarged. In the blood, the number of leukocytes is normal or close to normal, with a predominance or normal content of mature lymphocytes. The level of platelets is within the normal range, their number may decrease to 1 * 109 / l-1.4 * 109 / l in some patients after 7-10 years. More often, only a slight tendency to a decrease in the level of hemoglobin and the number of erythrocytes is revealed, reticulocytes fluctuate within 1.5-2%. Bone marrow biopsy reveals individual proliferates consisting of mature lymphocytes; histological studies of an enlarged lymph node and other affected organs help to verify the diagnosis. Malignancy of a lymphocytoma with transformation into lymphosarcoma or chronic lymphocytic leukemia is not mandatory, and if it does occur, it is often after many months or years.

Lymphoma non-Hodgkin- a heterogeneous group of diseases characterized by neoplastic proliferation of immature lymphoid cells that accumulate outside the bone marrow.

Code by international classification ICD-10 diseases:

  • C82- Follicular [nodular] non-Hodgkin's lymphoma
  • C83- Diffuse non-Hodgkin's lymphoma
Lymphosarcomatosis (Kundrat's disease)) - a generalized form of non-Hodgkin's lymphoma, characterized by multiple lesions of the lymph nodes, and subsequently - damage to the liver and spleen.

Frequency

Approximately 35,000 patients are diagnosed with non-Hodgkin's lymphoma each year in the United States.

Pathological classification. There are many histological classifications of the disease. To eliminate the contradictions between them in 1982, a classification was adopted National Institute Cancer: . Lymphoma low grade of malignancy. Small cell lymphocytic. Predominantly follicular (small cells with split nuclei). Follicular - mixed type(small cells with split nuclei and large cells). Lymphoma medium degree malignancy. Predominantly follicular large cell. Diffuse small cell with split nuclei. Diffuse mixed (small and large cell). Diffuse large cell. Lymphoma high degree of malignancy. Large cell. Lymphoblastic with curved nuclei. Small cell with unsplit nuclei (Burkett).

Types of lymphomas
. Low-grade lymphomas predominantly B-cell tumors. The intermediate type of lymphosarcoma includes both B-cell and some T-cell lymphomas. Immunoblastic lymphosarcomas are predominantly B-cell tumors, lymphoblastic lymphosarcomas are of T-cell origin. Most B-cell tumors are monoclonal and form - and -light chains of immunoglobulins.
. Follicular lymphomas(small cells with split nuclei) is the most characteristic histological type, accounting for about 40% of cases of all malignant lymphomas. This type is found mainly in stage III or IV of the disease with frequent damage to the bone marrow. The clinical picture is characterized by the absence of pain syndrome for many years.
. Follicular lymphomas, consisting of large and small cells with split nuclei, are found in 20-40% of patients. The bone marrow is usually affected.
. Diffuse large cell lymphomas characterizes the presence of large atypical lymphocytes with large nuclei.
. Immunoblastic lymphomas and other non-Hodgkin's lymphomas of a high degree of malignancy: plasmacytic, clear cell and polymorphic variants. Despite prompt and adequate treatment, these variants of lymphoma progress rapidly and lead to death. Burkett lymphoma(Burkett lymphosarcoma, lymphoma African) - malignant lymphoma, localized mainly outside the lymph nodes (upper jaw, kidneys, ovaries). High incidence in children in Africa and Asia (#113970, point mutations of the MYC, 8q24 genes, as well as  - (2p) and  - (22q) of light or heavy (14q32) immunoglobulin chains are often detected). Lymphadenopathy, hepatosplenomegaly, skin manifestations, hypercalcemia.

Non-Hodgkin's Lymphoma: Causes

Etiology

Immunodeficiencies. Long-term use of immunosuppressants (for example, after a kidney or heart transplant). Epstein-Barr virus is associated with the development of Burkett's lymphoma. Cytogenetic abnormalities (eg, chromosomal translocations).

Clinical picture

Proliferative syndrome: lymphadenopathy (enlargement of the affected lymph nodes); tumor syndrome: enlargement of the liver, spleen. Intoxication syndrome: fever, fatigue, weight loss and night sweats. Clinical manifestations depends on the location of the lymphosarcoma ( intestinal obstruction with abdominal localization; tracheal compression syndrome with lesions of the intrathoracic lymph nodes).
Stages of the disease and diagnosis. The principles of staging are similar to those for lymphogranulomatosis. The 4th stage of the disease is exhibited when the bone marrow (leukemization) and the central nervous system are involved in the pathological process. Stage setting. Biopsy of the lymph node and analysis of biopsy material. Hematological examination, including counting leukocyte formula, platelets, determination of uric acid content. Blood protein electrophoresis can rule out hypogammaglobulinemia and/or heavy chain disease. Collecting a complete anamnesis and medical examination with an emphasis on all groups of lymph nodes (primarily the von Waldeyer-Pirogov ring), as well as on the size of the liver and spleen. Bilateral biopsy and bone marrow aspiration. Radiological studies - X-ray of the chest, abdomen and pelvis, less often - bilateral lymphangiography lower extremities and pelvis. Other procedures are exploratory laparotomy, bone scintigraphy or radiography, endoscopy, and liver biopsy.

Non-Hodgkin's Lymphoma: Treatment Methods

Treatment

usually combined. As in the treatment of leukemia, various chemotherapy protocols are used.
. Chemotherapy. Intermediate and high-grade stage I and II lymphomas often respond well to combination chemotherapy (high doses of cyclophosphamide with methotrexate, vincristine, and often doxorubicin) with or without radiation therapy (80% to 90% of cases are cured). In case of damage to the central nervous system, cytostatics are administered endolumbally or into the ventricles of the brain.
. Irradiation. Non-Hodgkin's lymphomas are extremely radiosensitive. In a localized process, irradiation should be directed to the affected area (at a dose of 40 Gy). In disseminated lymphoma, radiation has a palliative effect, and also enhances healing effect chemotherapy. I stage of sluggish lymphomas. Long-term follow-up of patients with localized I and II stages of low-grade lymphoma, who received general irradiation of the lymph nodes, revealed the presence of a 10-year relapse-free period in 50% of cases (especially in young patients).

Features in children
.

Dominant age

- 5-9 years, the ratio of boys / girls - 2-2, 5/1.
. Features of the flow. Rapid progression of the tumor. The predominance of extranodal localization. Primary - generalized tumor.
. Localization. B - cell lymphomas - intestines (35%), nasopharynx (20%). T - cell lymphomas - mediastinum (25%), peripheral lymph nodes (15%).
.

Treatment

The main method is combined polychemotherapy. Radiation therapy apply only with damage to the central nervous system (locally).
.

Course and forecast

The 5-year survival rate with treatment reaches 80%.

Synonyms

Lymphosarcoma. Lymphoblastoma. Lymphoma malignant.

ICD-10. C82 Follicular [nodular] non-Hodgkin's lymphoma. C83 Diffuse non-Hodgkin's lymphoma