Chronic – the oncological disease which is followed by accumulation of atypical mature V-lymphocytes in peripheral blood, a liver, a spleen, lymph nodes and marrow. At initial stages it is shown limfotsitozy and a generalized limfoadenopatiya. When progressing a chronic limfoleykoz the gepatomegaliya and a splenomegaliya, and also anemia and the thrombocytopenia which are shown weakness, fatigue, petekhialny hemorrhages and the raised bleeding are observed. The frequent infections caused by decrease in immunity are noted. The diagnosis is established on the basis of laboratory researches. Treatment – chemotherapy, bone marrow transplantation.
Chronic – a disease from group of nekhodzhkinsky lymphoma. Is followed by increase in quantity of morphologically mature, but defective V-lymphocytes. Chronic is the most common form of gemoblastoz, makes a third of all leukoses diagnosed in the USA and the countries of Europe. Men suffer more often than women. The peak of incidence is the share of age of 50-70 years, in this period about 70% of total of chronic limfoleykoz come to light.
Patients of young age suffer seldom, up to 40 years the first a symptom of a disease arise at only 10% of patients. In recent years experts note some "rejuvenation" of pathology. The clinical current of a chronic limfoleykoz is very variable, possible both long lack of progressing, and extremely aggressive option with a lethal outcome within 2-3 years after diagnosis. There is a number of the factors allowing to predict the course of a disease. Treatment is performed by experts in the field of oncology and hematology.
Etiology and pathogenesis of a chronic limfoleykoz
The causes are finally not found out. Chronic it is considered the only leukosis with unconfirmed communication between development of a disease and adverse factors of the external environment (ionizing radiation, contact with cancerogenic substances). Experts consider that the major factor promoting development of a chronic limfoleykoz is hereditary predisposition. The typical chromosomal mutations causing damages of oncogenes at an initial stage of a disease are not revealed yet, however researches confirm the mutagen nature of a disease.
The clinical picture of a chronic limfoleykoz is caused limfotsitozy. Emergence of a large number morphologically mature, but immunological the defective V-lymphocytes incapable of ensuring humoral immunity becomes the reason of a limfotsitoz. Earlier considered that abnormal V-lymphocytes at a chronic limfoleykoz are long living cages and seldom are exposed to division. In the subsequent this theory was refuted. Researches showed that V-lymphocytes quickly breed. Daily in an organism of the patient 0,1-1% of total of atypical cages are formed. At different patients various clones of cages therefore chronic it is possible to consider as group of closely related diseases with the general etiopatogenez and similar clinical symptomatology are surprised.
When studying cages a big variety comes to light. In material shirokoplazmenny or uzkoplazmenny cages with young people or the wrinkled kernels, almost colourless or brightly painted granular cytoplasm can prevail. Proliferation of abnormal cages happens in pseudo-follicles – congestions of the leucemic cages which are settling down in lymph nodes and marrow. Cytosinging at a chronic limfoleykoz the autoimmune destruction of uniform elements of blood and suppression of proliferation of stem cells caused by increase in level of T-lymphocytes in a spleen and peripheral blood are the reasons. Besides, with killerny properties atypical V-lymphocytes can cause destruction of blood cages.
Classification of a chronic limfoleykoz
Taking into account symptoms, morphological features, speeds of progressing and reaction to therapy distinguish the following forms of a disease:
- Chronic with a good-quality current. The condition of the patient long remains satisfactory. Slow increase in quantity of leukocytes in blood is noted. From the moment of diagnosis before stable increase in lymph nodes there can pass several years or even decades. Patients keep working capacity and a habitual way of life.
- The classical (progressing) form of a chronic limfoleykoz. Leykotsitoz accrues within months, but not years. Parallel increase in lymph nodes is noted.
- Tumoral form of a chronic limfoleykoz. Distinctive feature of this form is unsharply expressed at the expressed increase in lymph nodes.
- Marrowy form of a chronic limfoleykoz. Comes to light progressing cytosinging in the absence of increase in lymph nodes, a liver and a spleen.
- Chronic with increase in a spleen.
- Chronic with a paraproteinemiya. Symptoms of one of above-mentioned forms of a disease in combination with monoclonal G-or M-gammapatiyey are noted.
- Prelimfotsitarny form of a chronic limfoleykoz. Distinctive feature of this form is existence of the lymphocytes containing nukleola in dabs of blood and marrow, samples of tissue of spleen and lymph nodes.
- Volosatokletochny leukosis. Cytosinging and a splenomegaliya in the absence of increase in lymph nodes come to light. At a microscopic research lymphocytes with a characteristic "young-looking" kernel and "uneven" cytoplasm with breaks, scalloped edges and sprouts in the form of hairs or fibers are found.
- T-cellular form of a chronic limfoleykoz. It is observed in 5% of cases. Is followed by leukemic infiltration terms. Usually quickly progresses.
Allocate three stages clinical to a stage of a chronic limfoleykoz: initial, the developed clinical manifestations and terminal.
Symptoms of a chronic limfoleykoz
At an initial stage pathology proceeds asymptomatically and can come to light only on blood tests. Within several months or years at the patient with a chronic limfoleykoz comes to light 40-50%. The quantity of leukocytes is brought closer to the upper bound of norm. In a usual state peripheral and visceral lymph nodes are not increased. In the period of infectious diseases lymph nodes can temporarily increase, and after recovery to decrease again. Stable increase in lymph nodes becomes the first sign of progressing of a chronic limfoleykoz, it is frequent – in combination with a gepatomegaliya and a splenomegaliya.
In the beginning cervical and axillary lymph nodes are surprised, then – knots in the field of a sredosteniye and an abdominal cavity, then – in inguinal area. At a palpation the mobile painless plotnoelastichesky educations which are not soldered to skin and nearby fabrics come to light. Diameter of knots at a chronic limfoleykoz can fluctuate from 0,5 to 5 and more centimeters. Large peripheral lymph nodes can vybukhat with formation of visible cosmetic defect. At significant increase in a liver, spleen and visceral lymph nodes the sdavleniye of internals which is followed by various functional violations can be observed.
Patients with a chronic limfoleykoz complain of weakness, causeless fatigue and decrease in working capacity. On blood tests increase in a limfotsitoz up to 80-90% is noted. The quantity of erythrocytes and platelets usually remains within norm, at some patients insignificant thrombocytopenia comes to light. At late stages of a chronic limfoleykoz weight reduction, night sweats and temperature increase to subfebrilny figures are noted. Disorders of immunity are characteristic. Patients often have catarrhal diseases, cystitis and uretrity. Tendency to suppuration of wounds and frequent formation of abscesses in hypodermic fatty cellulose is observed.
Serious infectious diseases become the reason of a lethal outcome at a chronic limfoleykoz often. The pneumonias which are followed by falling off of pulmonary fabric and gross violations of ventilation are possible. At some patients ekssudativny pleurisy which can be complicated by a gap or a sdavleniye of a chest lymphatic channel develops. One more frequent manifestation of the developed chronic limfoleykoz is shingles which in hard cases becomes generalized, taking all surface of skin, and sometimes and mucous membranes. Similar defeats can be observed at herpes and chicken pox.
Among other possible complications of a chronic limfoleykoz – the infiltration of a preddverno-ulitkovy nerve which is followed by disorders of hearing and noise in ears. In a terminal stage of a chronic limfoleykoz infiltration of brain covers, brain substance and nervous backs can be observed. On blood tests thrombocytopenia, hemolytic anemia and a granulotsitopeniye come to light. Transformation of a chronic limfoleykoz in Richter's syndrome – the diffusion lymphoma which is shown rapid growth of lymph nodes and formation of the centers outside lymphatic system is possible. About 5% of patients live up to development of a lymphoma. In other cases death comes from infectious complications, bleedings, anemia and a kakheksiya. At some patients with a chronic limfoleykoz the heavy renal failure caused by infiltration of a kidney parenchyma develops.
Diagnostics of a chronic limfoleykoz
In half of cases pathology is found incidentally, at inspection concerning other diseases or when carrying out planned survey. At diagnosis consider complaints, the anamnesis, data of objective survey, results of blood tests and immunophenotyping. Diagnostic criterion of a chronic limfoleykoz is increase in quantity of leukocytes in blood test to 5×109/ in combination with characteristic changes of an immunophenotype of lymphocytes. At a microscopic research of dab of blood small V-lymphocytes and Gumprekht's shadows come to light, it is possible – in combination with atypical or large lymphocytes. When immunophenotyping existence of cages with an abberantny immunophenotype and clonality is confirmed.
Definition of a stage of a chronic limfoleykoz is carried out on the basis of clinical displays of a disease and results of objective survey of peripheral lymph nodes. For scheduling of treatment and assessment of the forecast at a chronic limfoleykoz conduct cytogenetic researches. At suspicion appoint a biopsy to Richter's syndrome. For definition of the reasons cytosinging carry out a sternalny puncture of marrow with the subsequent microscopic research of a punktat.
Treatment and the forecast at a chronic limfoleykoz
At initial stages of a chronic limfoleykoz apply waiting tactics. To patients appoint inspection each 3-6 months. In the absence of signs of progressing are limited to observation. The indication to performing active treatment is increase in quantity of leukocytes twice and more within half a year. The main method of treatment of a chronic limfoleykoz is the chemotherapy. The combination of a rituksimab, a tsiklofosfamid and a fludarabin becomes the most effective combination of medicines usually.
At a persistent current of a chronic limfoleykoz appoint high doses of corticosteroids, carry out bone marrow transplantation. At patients of advanced age with heavy somatic pathology use of intensive chemotherapy and bone marrow transplantation can be complicated. In similar cases carry out monochemotherapy hlorambutsily or apply this medicine in combination with rituksimaby. At a chronic limfoleykoz with an autoimmune tsitopeniya appoint Prednisolonum. Treatment is performed before improvement of a condition of the patient, at the same time duration of a course of therapy makes not less than 8-12 months. After stable improvement of a condition of the patient treatment is stopped. The indication for renewal of therapy is the clinical and laboratory symptomatology demonstrating progressing of a disease.
Chronic is considered as almost incurable the current disease with rather satisfactory forecast is long. In 15% of cases the aggressive current with bystry increase of a leykotsitoz and progressing of clinical symptomatology is observed. The lethal outcome at this form of a chronic limfoleykoz comes within 2-3 years. In other cases slow progressing is noted, average life expectancy from the moment of diagnosis fluctuates from 5 to 10 years. At a good-quality current the term of life can make several decades. After passing of a course of treatment improvement is observed at 40-70% of patients with a chronic limfoleykoz, however full remissions come to light seldom.