Nekhodzhkinsky lymphoma – the tumoral diseases of lymphatic system presented by malignant B-and T-cellular lymphoma. Primary center can arise in lymph nodes or other bodies and further spread in the limfogenny or hematogenic way. The clinic of lymphoma is characterized by a limfadenopatiya, symptoms of defeat of this or that body, feverish a syndrome. Diagnostics is based on kliniko-radiological yielded, results of a research of a gemogramma, bioptat of lymph nodes and marrow. Antineoplastic treatment includes courses of polychemotherapy and radiation therapy.
Nekhodzhkinsky lymphoma (NHL, limfosarkoma) – malignant limfoproliferativny tumors, various on morphology, clinical signs and a current, excellent according to the characteristics from Hodzhkin's (limfogranulematoza) lymphoma. Depending on the place of emergence of primary center of a gemoblastoza are divided into leukoses (tumoral damages of marrow) and a lymphoma (a tumor of lymphoid fabric with primary extra marrowy localization). On the basis of distinctive morphological features of a lymphoma, in turn, are subdivided on hodzhkinsky and nekhodzhkinsky; in hematology carry to number of the last In - and T-cellular lymphoma. Nekhodzhkinsky lymphoma meet in all age groups, however more than a half of cases is diagnosed by a limfosarok for persons 60 years are more senior. The average value of incidence among men makes 2-7 cases, among women – 1-5 cases on 100 000 population. In recent years the tendency to the progressing increase in incidence is traced.
The etiology a limfosarok is authentically unknown. Moreover, the reasons of lymphoma of various histologic types and localizations significantly vary. Now it is more correct to speak about the risk factors increasing probability of development of a lymphoma which are at the moment well studied. Influence of one etiofaktor is expressed considerably, the contribution of others to an etiology of lymphoma is very insignificant. Treat such adverse prerequisites:
- Infections. The greatest cytopathogenic effect on lymphoid cages the human immunodeficiency virus (HIV), hepatitis C, T-limfotropny a virus of 1 type has. Connection of infection with Epstein-Barre's virus with development of a lymphoma of Berkitt is proved. It is known that Helicobacter pylori infection associated with stomach ulcer of a stomach can cause development of a lymphoma of the same localization.
- Defects of immunity. The risk of emergence of lymphoma increases at the congenital and acquired immunodeficiencies (AIDS, Viskotta-Aldrich's syndrome, Louis Bar, the H-linked limfoproliferativny syndrome, etc.). At the patients receiving immunosupressivny therapy concerning transplantation of marrow or bodies, the probability of development of NHL increases at 30-50 times.
- Associated diseases. The increased risk of incidence of NHL is noted among patients with rheumatoid arthritis, a red volchanka that can be explained with both immune violations, and use of immunosupressivny medicines for treatment of these states. The lymphoma of a thyroid gland usually develops against the background of an autoimmune tireoidit.
- Toxic influence. Relationship of cause and effect between limfosarkoma and the previous contact with chemical carcinogens (benzene, insecticides, herbicides), UF-radiation, performing radiation therapy concerning an oncological disease is traced. The cytostatic medicines applied to chemotherapy have direct tsitopatichesky effect.
Pathological it is initiated by this or that onkogenny event causing violation of a normal cellular cycle. Two mechanisms - activation of oncogenes or suppression of tumoral supressor (antioncogenes) can be involved in it. The tumoral clone at NHL in 90% of cases is formed of V-lymphocytes, is extremely rare – from T-lymphocytes, NK-of cages or undifferentiated cages. Certain chromosomal translocations which lead to suppression of apoptosis, loss of control over proliferation and a differentiation of lymphocytes at any stage are characteristic of various types of lymphoma. It is followed by emergence of a clone of blastny cages in lymphatic bodies. Lymph nodes (peripheral, mediastinalny, mezenterialny, etc.) increase in sizes and can break function of nearby bodies. At infiltration of marrow develops cytosinging. Growth and metastasis of tumoral weight is followed by a kakheksiya.
Limfosarkoma, initially developing in lymph nodes are called nodalny, in other bodies (palatal and pharyngeal almonds, salivary glands, a stomach, spleen, intestines, brain, lungs, skin, a thyroid gland, etc.) - ekstranodalny. Are divided by structure of tumoral fabric of NHL on follicular (nodulyarny) and diffusion. Are classified by rates of progressing of a lymphoma on indolentny (with slow, rather favorable current), aggressive and highly aggressive (with rapid development and generalization). In the absence of treatment patients with indolentny lymphoma live on average 7 – 10 years, with aggressive – from several months to 1,5-2 years.
Modern classification contains over 30 different types a limfosarok. The most part of tumors (85%) comes from V-lymphocytes (V-cellular lymphoma), other of T-lymphocytes (T-cellular lymphoma). In these groups there are various subtypes of nekhodzhkinsky lymphoma. The group of V-cellular tumors includes:
- diffusion V-krupnokletochnuyu a lymphoma – the most widespread histologic type a limfosarok (31%). It is characterized by aggressive growth, despite it almost in half of cases gives in to full treatment.
- follicular lymphoma – its frequency makes 22% of number of NHL. The current is indolentny, however transformation in an aggressive diffusion lymphoma is possible. The forecast of 5-year survival – 60-70%.
- melkokletochny lymphocytic lymphoma and chronic lymphocytic leukosis – close types of NHL to which share 7% of their number fall. The current is slow, but badly giving in to therapy. The forecast is variable: in one cases of a limfosarkom develops within 10 years, in others – at a certain stage turns into a fast-growing lymphoma.
- the lymphoma of mantle cages – in structure of NHL is made by 6%. The five-year boundary of survival overcomes only 20% of patients.
- V-cellular lymphoma from cages of a marginal zone – share on ekstranodalny (can develop in a stomach, thyroid, salivary, mammary glands), nodalny (develop in lymph nodes), splenic (with localization in a spleen). Differ in slow local growth; at early stages well give in to treatment.
- V-cellular mediastinalny lymphoma – meets seldom (in 2% of cases), however unlike other types strikes mainly young women of 30-40 years. Due to the rapid growth causes a compression of bodies of a sredosteniye; recovers in 50% of cases.
- Valdenstrem's makroglobulinemiya (limfoplazmotsitarny lymphoma) – it is diagnosed for 1% of patients with NHL. It is characterized by IgM hyperproduction by tumor cells that leads to increase in viscosity of blood, vascular thromboses, ruptures of capillaries. Can have as rather good-quality (with survival to 20 years), and transient development (with death of the patient within 1-2 years).
- volosatokletochny leukosis – very rare type of a lymphoma which is found at elderly people. The course of a tumor slow, not always demanding treatment.
- Berkitt's lymphoma – about 2% of NHL fall to its share. In 90% of cases the tumor affects young men up to 30 years. Growth of a lymphoma of Berkitt aggressive; the intensive chemotherapy allows to achieve treatment of a half of patients.
- lymphoma of the central nervous system – primary defeat of TsNS can affect a head or spinal cord. Is associated with HIV infection more often. Five-year survival makes 30%.
Nekhodzhkinsky lymphoma of the T-cellular origin are presented:
- T-limfoblastnoy a lymphoma or a leukosis from cages predecessors – meets frequency of 2%. Differ among themselves with quantity of blastny cages in marrow: at 25% - as a leukosis. Average age of the diseased – is diagnosed 25 years mainly for young people. The worst forecast has T-limfoblastny a leukosis, the treatment indicator at which does not exceed 20%.
- the peripheral T-cellular lymphoma including a skin lymphoma (Cesaria's syndrome, fungoid mycosis), an angioimmunoblastny lymphoma, an ekstranodalny lymphoma from natural killers, a lymphoma with an enteropatiya, a pannikulitopodobny lymphoma of hypodermic cellulose, a krupnokletochny anaplastichesky lymphoma. Current of the most part of T-cellular lymphoma bystry, and failure.
Options of clinical manifestations of NHL strongly vary depending on localization of primary center, prevalence of tumoral process, histologic type of a tumor and so forth. All manifestations by a limfosarok keep within three syndromes: limfadenopatiya, fevers and intoxications, ekstranodalny defeat. In most cases increase in peripheral lymph nodes is the first sign of NHL. In the beginning they remain elastic and mobile, later merge in extensive conglomerates. At the same time lymph nodes of one or many areas can be surprised. At formation of the svishchevy courses it is necessary to exclude actinomycosis and tuberculosis.
Such nonspecific symptoms a limfosarok as fever without the obvious reasons, night perspiration, loss of weight, an adynamy in most cases indicate the generalized nature of a disease. Among ekstranodalny defeats nekhodzhkinsky lymphoma of a ring of Pirogov-Valdeyera, a GIT, brain dominate, the mammary gland, bones, a parenchyma of lungs, etc. bodies are surprised less often. The nasopharynx lymphoma at an endoscopic research has an appearance of a tumor of light pink color with hilly contours. Often sprouts a maxillary and trellised bosom, an orbit, causing difficulty of nasal breath, a rinofoniya, decrease in hearing, .
Primary limfosarkoma of a small egg can have a smooth or hilly surface, elastichesky or stony density. In certain cases scrotum hypostasis, a skin ulceration over a tumor, increase inguinal lymph nodes develops. Lymphoma of a small egg are predisposed to an early dissemination with defeat of the second small egg, TsNS, etc. The lymphoma of a mammary gland at a palpation is defined as accurate tumoral knot or diffusion consolidation of a breast; retraction of a nipple is uncharacteristic. At damage of a stomach the clinical picture reminds stomach cancer, being followed by pains, nausea, appetite loss, weight reduction. Abdominal limfosarkoma can prove partial or full intestinal impassability, peritonitis, a malabsorption syndrome, belly-aches, ascites. The lymphoma of skin is shown by an itch, small knots and consolidation of reddish-crimson color. Primary defeat of TsNS is more characteristic of patients with AIDS – a current of a lymphoma of this localization are followed by focal or meningealny symptomatology.
Presence of considerable tumoral weight can cause a sdavleniye of bodies with development of zhizneugrozhashchy states. At damage of mediastinalny lymph nodes the compression of a gullet and trachea, VPV sdavleniye syndrome develops. The increased intra belly and zabryushinny lymph nodes can cause the phenomena of intestinal impassability, a limfostaz in the lower half of a trunk, mechanical jaundice, a compression of a mochetochnik. Germination of walls of a stomach or intestines is dangerous by developing of bleeding (in case of an arrosion of vessels) or peritonitis (at a contents exit in an abdominal cavity). Immunosupression causes susceptibility of patients to the infectious diseases posing a threat for life. Early limfogenny and hematogenic metastasis in a head and spinal cord, a liver, bones is characteristic of lymphoma of high degree of a zlokachestvennost.
Questions of diagnostics of nekhodzhkinsky lymphoma are in competence of onkogematolog. As clinical criteria of a limfosarkoma serve increase in one or several groups of lymph nodes, intoxication phenomena, ekstranodalny defeats. Confirmation of the estimated diagnosis requires carrying out morphological verification of a tumor and tool diagnostics:
- Research of a cellular substratum of a tumor. Diagnostic operations are carried out: a punktsionny or ekstsizionny biopsy of lymph nodes, a laparoscopy, a torakoskopiya, an aspiration puncture of marrow with the subsequent immunohistochemical, cytologic, cytogenetic and other researches of diagnostic material. Except diagnostics, establishment of structure of NHL is important for the choice of tactics of treatment and definition of the forecast.
- Visualization methods. Increase in mediastinalny and intra belly lymph nodes is found by means of ultrasonography of a sredosteniye, a X-ray analysis and KT of a thorax, an abdominal cavity. Spleens, mammary glands, a thyroid gland, bodies of a scrotum, gastroscopy enter an inspection algorithm according to indications ultrasonography of lymph nodes, a liver. For the purpose of a stadirovaniye of a tumor MRT of internals is carried out; in identification of metastasises the limfostsintigrafiya, a stsintigrafiya of bones are informative.
- Laboratory diagnostics. It is directed to assessment of risk factors and function of internals at lymphoma of various localizations. In risk group definition VICh-antigena, anti-HCV is made. Change of peripheral blood () is characteristic of a leykemization. In all cases the biochemical complex including pechenochyony enzymes, LDG, uric acid, creatinine, etc. indicators is investigated. B2-microglobulin can serve as a peculiar onkomarker of NHL.
It is necessary to differentiate nekhodzhkinsky lymphoma with limfogranulematozy, the metastatic cancer, lymphadenites arising at a tulyaremiya, a brucellosis, syphilis, tuberculosis, toxoplasmosis, an infectious mononukleoz, flu, hard currency, etc. At lymphoma of concrete localizations consultations of profile experts are held: otorhinolaryngologist, gastroenterologist, mammologist, etc.
treatment should be carried out only under the supervision of a doctor. Self-treatment is unacceptable!!!
Options of treatment of nekhodzhkinsky lymphoma include an operational method, radiation therapy and chemotherapy. The choice of a technique is defined by morphological type, prevalence, localization of a tumor, safety and age of the patient. In modern haemato-oncology the protocols of treatment by a limfosarok which are based on use are adopted:
- Chemotherapy. Most often treatment of lymphoma is begun with a polychemotherapy course. This method can be independent or be combined with radiation therapy. The combined himioluchevy therapy allows to reach longer remissions. Treatment continues before achievement of full remission then carrying out 2-3 more consolidating courses is necessary. Inclusion in cycles of treatment of hormonal therapy is possible.
- Surgical interventions. It is usually applied at the isolated defeat of any body, a thicket - a GIT. Whenever possible operations have radical character – the expanded and combined resections are carried out. In the started cases, at threat of perforation of hollow bodies, bleedings, impassability of intestines can be carried out cytoreductive interventions. Surgical treatment is surely supplemented with chemotherapy.
- Radiation therapy. As monotherapy of lymphoma it is applied only at the localized forms and low degree of a zlokachestvennost of a tumor. Besides, radiation can be used also as a palliative method at impossibility of carrying out other options of treatment.
- Additional schemes of treatment. From alternative methods the immunochemotherapy with use of interferon, monoclonal antibodies well proved. For the purpose of consolidation of remission transplantation of autologichny or allogenny marrow and introduction of peripheral stem cells is applied.
Forecast and prevention
The forecast at nekhodzhkinsky lymphoma is various, depends, mainly, on histologic type of a tumor and a stage of identification. At locally-spread forms long-term survival averages 50-60%, at generalized - only 10-15%. Adverse predictive factors age 60 years, the III-IV stages of an onkoprotsess, involvement of marrow, existence of several ekstranodalny centers serve more senior. At the same time, the PHT modern protocols in many cases allow to achieve long-term remission. Prevention of lymphoma correlates with the known reasons: it is recommended to avoid infection with cytopathogenic viruses, toxic influences, excessive insolation. With risk factors it is necessary to undergo regular inspection.