Nekhodzhkinsky lymphoma

Description:

Nekhodzhkinsky lymphoma – the whole group from more than 30 related diseases, not having characteristics of a hodzhkinsky disease. A lymphoma – a kind of cancer affecting the lymphatic system consisting of lymph nodes (the small closed accumulations of the lymphocytes) combined by system of small vessels.

The standardized indicators of incidence lympho-and reticulosarcomas fluctuate within 2-6,9 at men, 0,9-5 at women.

Get sick with Nekhodzhkinsky lymphoma of the man considerably more often than women, their age by the time of establishment of the diagnosis fluctuates in big limits.

Symptoms of the Nekhodzhkinsky lymphoma:

For all morphological options of nekhodzhkinsky lymphoma equally frequent defeat both lymph nodes in general, and their separate groups, a lymphoid ring of Valdeyer and digestive tract is. More frequent primary damage of retroperitoneal lymph nodes and an abdominal cavity, bones and soft tissues is observed at lymphoblastoid, spleens - at pro-lymphocytic options. Pathological process, irrespective of morphological option of a disease, in bigger honor of cases extends at first not the zones adjacent to lymph nodes. Defeat of adjacent groups of lymph nodes takes place at lymphoblastoid option more often.

Early extranodal innidiation, innidiation in marrow, involvement in pathological process of a liver and spleen to several bowl are noted at pro-lymphocytic option, and defeat bone a brain and a leykemization meet in the presence of cells with the roundish and split kernel more often. At the same time at blast options involvement of marrow and increase of the sizes of lymph nodes occur more early.

The greatest distinctions between morphological options are noted at survival assessment. Five-year survival at pro-lymphocytic option with the split and roundish kernel makes 70 and 53% of small cells respectively. At prolimfotsitarno-lymphoblastoid option from large cells with the split kernel indicators of survival approach that at blast options and make 14-21 month.

Survival indicators in the I-II stages of nekhodzhkinsky lymphoma with high degree of a zlokachestvennost at primary gastrointestinal tract disease considerably surpass observed in the general group of patients with these options.

Primary nekhodzhkinsky lymphoma of a spleen - rare localization (less than 1%) whereas its involvement in pathological process often (40-50%) meets at lymphosarcomas. Slightly more often primary damage of a spleen is found at pro-lymphocytic option. More often at a lymphoma of a spleen involvement in pathological process of marrow is stated. At the same time at lymphoblastoid option metastasises from a spleen are more often localized in abdominal lymph nodes.

The most frequent damage of lungs is found at nekhodzhkinsky lymphoma of low degree of a zlokachestvennost. The forecast and at this primary localization is defined by morphological option. Defeat of a nervous system is stated, as a rule, at blast options of nekhodzhkinsky lymphoma.

The Nodulyarny type of nekhodzhkinsky lymphoma and limits of any histologic type is characterized by more favorable disease. At lymphocytic option, despite bystry generalization of process, rather high-quality current is also noted.

The Kliniko-gematologichesky picture at separate morphological options of diffusion lymphosarcomas has the features. So, quite early generalization of process is characteristic of lymphocytic option. Unlike a chronic lymphoid leukosis it is possible to track quite often the sequence of involvement and pathological process of various groups of lymph nodes, at a histologic research of marrow the knotty or knotty and diffusion type of defeat (and difference from the diffusion nature of infiltration comes to light at a chronic lymphoid leukosis).

Generalization of process and average occurs in 3-24 months. Damage of marrow can be revealed also at a normal gemogramma (at 47% of patients it and the moment of establishment of the diagnosis is not changed), at a part of patients the lymphocytopenia comes to light. Despite early generalization and involvement in process of marrow, the forecast of a disease at this option rather favorable (to 75% of patients there live more than 5 years).

The T-cellular option of a lymphosarcoma differs in a clinical and hematologic originality: a splenomegaly, generalized increase in lymph nodes, infiltrates in lungs, damage of skin. Primary center is T-dependent paracortical облвсть lymph nodes. The high lymphocytosis in blood is noted, kernels of bigger honor of lymphocytes are overwound. Average life expectancy at this rare option short - 10 months.

At rare limfoplazmotsitarny cytologic option clinical syndromes of a course of a disease are defined by localization of a tumor, prevalence degree process, is frequent - the number of IgM in blood serum.

The pro-lymphocytic option is found in 45-51% of all cases of lymphosarcomas. At it increase in occipital, parotid, subnodal, lymph nodes often comes to light. Without watching not rvnny generalization and a frequent leykemization (in 25-45%) process, at this option five-year survival of patients makes 63-70%. At a prolimfotsitorno-lymphoblastoid sub-option the forecast less favorable.

The lymphoblastoid option, quite heterogeneous on morphological (with the twisted, not twisted kernel, macro - microforms) and immunological (T - and the V-phenotype) to characteristics, most often occurs at children. Lymph nodes of various localization are surprised. The disease differs in prompt rates growth of tumors and involvement е process of new anatomic zones. More often than at other lymphosarcomas, in a gemogramma the initial cytopenia, the T phenotype of lymphocytes obnvruzhivvtsya.

Berkitt's lymphoma of the V-cellular origin is carried to lymphoblastoid type of a lymphosarcoma. Its classical option is shown preferential by damage of bones (especially a mandible), kidneys, ovaries, lymph nodes of retroperitoneal areas, easy, parotid sialadens. Marrow is seldom involved in process. At the localized forms the forecast favorable with long remissions up to full treatment. The most widespread type of the T-lymphoblastoid lymphoma - "protimotsitny". In most cases the mediastinum is surprised, metastasises in the central nervous system, lungs come to light; in 50% of cases - a leykemization. The disease comes to light at boys of the first 5 years of life and teenagers of 13-16 years more often.

The immunoblast lymphosarcoma (the B-phenotype prevails) can develop as primary tumor of digestive tract, lymph nodes, Valdeyer's rings, etc. Often the cytopenia, a leykemization - in rare instances comes to light. The disease progresses quickly, five-year survival of patients makes 21-32%, however removal of a solitary tumor can promote long-term remission and even treatment. The immunoblast lymphosarcoma as secondary process is described at a multiple myeloma, Valdenstrem's macroglobulinemia, other limfoproliferativny diseases.

Fungoid mycosis represents a malignant lymphoid tumor, initially always arising in upper layers of a derma, consisting from polymorphic T-helperov. The nonspecific inflammation can be the first display of a disease. The diagnosis is verified according to a histologic, cytochemical research (lymphoid cells give positive reaction to acid phosphatase, beta glucuronidase and acid nonspecific esterase). There is a point of view that the early, chronic phase of a disease can be reactive, and "lymphoblastoid" represents true malignant transformation. Cesaria's syndrome which is characterized by emergence in a gemogramma of lymphoid cells from a mozgovidny look a kernel is considered as a leukemic phase of fungoid mycosis.

The histiocytic option of malignant nekhodzhkinsky lymphoma is a big rarity. His clinical picture is various. Metastasises can be found in many bodies. Leykemization and damage of marrow arise seldom, the cytopenia often comes to light.

Nosological accessory of the allocated new forms remains diskutabelny. So, an independent form suggest to consider Lennert's lymphoma described originally as unusual option of a lymphogranulomatosis with the high content of epithelial cells. Lack of typical cells of Berezovsky - Shternberg, fibrosis, the high content of immunoblasts, plasmocytes, transitions to a lymphosarcoma formed the basis for an otgranicheniye of this disease from a lymphogranulomatosis and its allocation under the name "Lennert's lymphoma" (a malignant lymphoma with the high content of epithelioid histiocytes, a limfoepitelialny lymphoma, an epitelioidnokletochny lymphoma). Feature of clinical manifestations of a lymphoma of Lennert is frequent defeat of palatine tonsils of lymph nodes, advanced age of patients, existence of a polyclonal gammapathy and allergic skin rashes in the anamnesis.

Suggest to carry the angioimmunoblastny limfoadenopatiya which is also described in recent years with a disproteinemia (a lymphogranulomatosis of X) to nekhodzhkinsky lymphoma. Clinically the disease is shown by fever, loss of body weight, skin rashes, a generalized limfoadenopatiya, is frequent in combination with gepato-and a splenomegaly, a resistant hyperglobulinemia, sometimes hemolysis signs. The triad is histologically characteristic: growth of small vessels, proliferation of immunoblasts, adjournment of PAS positive amorphous masses in walls of vessels. The quantity of eosinophils and histiocytes fluctuates, however sometimes the number of the last is considerably increased. Existence of colossal cells, the small centers of a necrosis is possible. A number of researchers regard the above described changes not as a malignant lymphoma and as reactive, connected with disturbances in system of V-lymphocytes.

Lymphocytes can be localized in various bodies and fabrics (a spleen, lymph nodes, a stomach, lungs, skin, etc.). The disease progresses slowly. For a long time the spleen, lymph nodes of the normal sizes is slightly increased or are slightly increased. In blood quantity of leukocytes, normal or close to norm, with dominance or the normal maintenance of end-stage lymphocytes. Level of thrombocytes within norm, the quantity them can at some patients in 7-10 years decrease to 1*109/l-1,4*109/l. More often only the insignificant tendency to decrease in level of hemoglobin and quantity of erythrocytes comes to light, reticulocytes fluctuate within 1,5-2%. In a bioptata of marrow the separate proliferata consisting of end-stage lymphocytes are found; histologic researches of the increased lymph node, other struck bodies help to verify the diagnosis. Ozlokachestvleny lymphocytomas with transformation in a lymphosarcoma or a chronic lymphoid leukosis is not obligatory and if it also arises, then it is frequent in many months or years.

Reasons of the Nekhodzhkinsky lymphoma:

The etiology of an origin of a nekhodzhkinsky lymphoma is unknown. It is considered that the reason consists in penetration of a viral infection into a human body or owing to suppression of immune system, especially after organ transplantation. A virus Epstein-Burra possibly causes Burkett's lymphoma, one of kinds of a nekhodzhkinsky lymphoma.

Treatment of the Nekhodzhkinsky lymphoma:

Therapy of lymphosarcomas is defined first of all by morphological option (zlokachestvennost degree), the nature of prevalence of pathological process (stage), the sizes and localization of a tumor, age of the patient, existence of other diseases.

Fundamental method of treatment of nekhodzhkinsky lymphoma is the polychemotherapy capable to provide development of kliniko-hematologic remission, its consolidation and antirecurrent treatment. Local radiation therapy is in most cases justified in a combination with chemotherapy or as palliative radiation of tumoral educations. As the independent method radiation therapy at nekhodzhkinsky lymphoma can be used only in accurately proved I stage of a disease at nekhodzhkinsky lymphoma of low degree of a zlokachestvennost, when involving in process of bones.

After local radiation therapy or an operational oncotomy full remission remains at many patients more than 5-10 years. The disease progresses slowly at process generalization.

By the elderly patient with nekhodzhkinsky lymphoma of low degree of a zlokachestvennost, and also in the presence of serious associated diseases the monochemotherapy can be applied (Chlorbutinum, pafentsit, Cyclophosphanum, etc.). At primary damage of a spleen irrespective of morphological option of a disease carry out a splenectomy with the subsequent course of chemotherapy. At the isolated damage of a stomach include its resection in the program of the combined treatment. At damage of skin use the chemotherapy including Prospidinum and Spirobrominum.

In the III-IV stages of a disease at aggressive nekhodzhkinsky lymphoma induction of remission is carried out by polychemotherapy courses with the subsequent consolidating treatment. On area of the most aggressive tumoral growth local radiation therapy can be applied, it is effective also at primary lymphosarcomas of a pharyngeal ring. However, as it is impossible to exclude a possibility of generalization of process, it is reasonable to combine beam treatment with chemotherapy.

At generalized stages of nekhodzhkinsky lymphoma of low degree of a zlokachestvennost apply the TsOP, TsOPP, BATsOP programs, etc.

At blast options it is reasonable to persons of young age to appoint programs of treatment of acute lymphoblastoid leukoses and prevention of a neuroleukosis.

Results of cytostatic therapy of nekhodzhkinsky lymphoma depend also on timely applied means of hematologic resuscitation" immunocorrection and antibacterial therapy.

As a hemopoiesis at a part of patients some time remains safe, there is a possibility of carrying out polychemotherapy in out-patient conditions that at the accurate organization of dispensary observation facilitates medical process, allows patients to avoid heavy psychological influences of oncological and hematologic hospitals.
After achievement of kliniko-hematologic remission within 2 years with intervals in 3 months antirecurrent therapy is performed. At achievement of permanent remission treatment stops.

In the last 10 years survival at aggressive nekhodzhkinsky lymphoma considerably increased thanks to the programs including 5-6 cytostatic drugs. Modern therapy promotes achievement of full remissions at 70-80% and 5-year bezretsidivny survival at 65-70% of patients.

In recent years clinical experience on use at nekhodzhkinsky lymphoma of transplantations of allogenic and autologous marrow is accumulated.

Lymphocytomas throughout a long time can not demand special treatment. In their therapy the operational oncotomy, a monokhimiolecheniye (Cyclophosphanum, Chlorbutinum), glucocorticosteroids, antihistaminic drugs and other means are applied according to indications. In transformation cases in a lymphosarcoma or a chronic lymphoid leukosis appoint the programs of the combined cytostatic therapy which are used at these diseases.

The forecast of a disease depends on a type of a lymphoma, a stage of a disease, its distribution, reaction to treatment, etc. Correctly picked up treatment increases forecasts for recovery.