Chronic leukosis – primary tumoral disease of system of blood formation as which substratum the mature and ripening cages of a myeloid or lymphoid row act. Various forms of a chronic leukosis proceed with prevalence intoksikatsionny (weakness, artralgiya, ossalgiya, anorexia, weight loss), trombogemorragichesky (bleeding, thromboses of various localization), limfoproliferativny syndromes (increase in limfouz, a splenomegaliya, etc.). Crucial importance in diagnosis of a chronic leukosis belongs to research OAK, bioptat of marrow and lymph nodes. Treatment of chronic leukoses is carried out by methods of chemotherapy, radiation therapy, an immunotherapy, transplantation of marrow is possible.
Chronic leukosis – the chronic limfoproliferativny and miyeloproliferativny diseases which are characterized by excess increase in quantity of the haematogenic cages keeping ability to a differentiation. Unlike sharp leukoses at which there is a proliferation of the low-differentiated haemo poetic cages at chronic leukoses the tumoral substratum is presented by the ripening or mature cages. The long stage of a benign monoclonal tumor is characteristic of all types of chronic leukoses.
Chronic leukoses affect mainly adult at the age of 40-50 years; men are ill more often. About 30% of cases, a chronic myeloid leukosis - 20% of all forms of leukemia fall to the share of a chronic lymphoid leukosis. Chronic in hematology it is diagnosed twice more often than chronic myeloid leukemia. Leukoses at children proceed in chronic option extremely seldom - in 1-2% of cases.
Reasons of a chronic leukosis
The true reasons leading to development of a chronic leukosis are unknown. Now the greatest recognition was gained by the virus and genetic theory of gemoblastoz. According to this hypothesis, some types of viruses (among which – a virus Ebshteyna-Barre, retroviruses, etc.) are capable to get into unripe haematogenic cages and to cause their free division. Also the heredity role in an origin of leukoses as it is for certain known that the disease quite often has family character is not called in question. Besides, chronic myeloid leukemia in 95% of cases is associated with anomaly of the 22nd chromosome (Philadelphian or PH chromosome) which fragment of a long shoulder on the 9th chromosome.
As the most significant contributing factors to different types and forms of chronic leukoses impacts on an organism of high doses of radiation, x-ray radiation, production chemical vrednost (varnishes, paints, etc.), medicines (salts of gold, antibiotics, tsitostatik), a long experience of smoking act. The risk of development of a chronic lymphocytic leukosis increases at long contact with herbicides and pesticides, and a chronic myeloid leukosis – at radiation exposure.
In pathogenesis of a chronic limfoleykoz the significant role belongs to immunological mechanisms – its frequent combination to autoimmune hemolytic anemia and thrombocytopenia, collagenases testifies to it. At the same time, at most of patients with chronic leukoses it is prichinno not possible to reveal significant factors.
Classification of a chronic leukosis
Depending on an origin and a cellular substratum of a tumor chronic leukoses share on lymphocytic, miyelotsitarny (granulotsitarny) and monocyte-derived. Treat group of chronic leukoses of a lymphocytic origin: chronic , Cesaria's disease ( skin), volosatokletochny leukosis, paraproteinemichesky gemoblastoza (miyelomny disease, Valdenstrem's makroglobulinemiya, pulmonary diseases of chains, diseases of heavy chains).
Chronic leukoses of a miyelotsitarny origin include the following forms: chronic myeloid leukemia, eritremiya, true politsitemiya, chronic , etc. Treat chronic leukoses of a monocyte-derived origin: chronic monocyte-derived leukosis and gistiotsitoza.
Tumoral process at a chronic leukosis takes place two stages in the development: monoclonal (good-quality) and polyclonal (malignant). The course of a chronic leukosis is conditionally subdivided into 3 stages: initial, developed and terminal.
Symptoms of a chronic myeloid leukosis
In an initial stage of chronic myeloid leukemia clinical manifestations are absent or are not specific, hematologic changes come to light incidentally at blood test. In the preclinical period increase of weakness, an adinamiya, perspiration, a subfebrilitet, pains in the left podreberye is possible.
Transition of a chronic myeloid leukosis to the developed stage is marked the progressing giperplaziy spleen and a liver, the anorexia, weight loss expressed by bone pains and artralgiya. Formation of leukemic infiltrates on skin, mucous oral cavities (leukemic periodontitis), a GIT is characteristic. The hemorrhagic syndrome is shown gematuriy, menorragiy, by a metrorragiya, bleedings after extraction of teeth, bloody ponosa. In case of accession of a secondary infection (pneumonia, tuberculosis, sepsis, etc.) the temperature curve gains gektichesky character.
The terminal stage of a chronic myeloid leukosis proceeds with a sharp aggravation of all symptoms and the expressed intoxication. Badly giving in therapies and life-endangering state – blastny crisis when because of sharp increase in quantity of blastny cages the course of a disease becomes similar to a sharp leukosis can develop in this period. The aggressive symptomatology is characteristic of blastny crisis: skin leykemida, heavy bleedings, secondary infections, high temperature, the rupture of a spleen is possible.
Symptoms of a chronic lymphoid leukosis
Long time the only sign of a chronic limfoleykoz can be to 40-50%, insignificant increase in one-two groups of lymph nodes. During the developed period lymphadenitis takes the generalized form: not only peripheral, but also mediastinalny, mezenterialny, zabryushinny knots increase. There is spleno-also a gepatomegaliya; perhaps sdavleny a holedokha the increased lymph nodes with development of jaundice, and also the top hollow vein with development of hypostases of a neck, person, hands (VPV syndrome). Persistent ossalgiya, a skin itch, recurrent infections disturb.
Weight of the general condition of patients with a chronic lymphoid leukosis is caused by progressing of intoxication (weakness, perspiration, fever, anorexia) and an anemichesky syndrome (dizziness, short wind, heartbeat, unconscious states).
The terminal stage of a chronic lymphoid leukosis is characterized by accession of hemorrhagic and immunodeficient syndromes. Heavy intoxication develops in this period, there are hemorrhages under skin and mucous, nasal, desnevy, uterine bleedings. The immunodeficiency caused by inability of functionally unripe leukocytes to perform the protective functions is shown by a syndrome of infectious complications. At patients with a chronic limfoleykoz pulmonary infections (bronchitis, bacterial pneumonia, tubercular pleurisy), fungal damages of skin and mucous, abscesses and phlegmons of soft fabrics, pyelonephritises, a herpetic infection, sepsis are frequent.
Dystrophic changes of internals, kakheksiya, renal failure accrue. The lethal outcome at a chronic lymphoid leukosis comes from heavy infectious and septic complications, bleedings, anemia, exhaustion. Transformation of a chronic limfoleykoz in a sharp leukosis or limfosarky is possible (a nekhodzhkinsky lymphoma).
Diagnosis of a chronic leukosis
The estimated diagnosis is established on the basis of the analysis of a gemogramma with which results the patient has to be immediately directed to the hematologist. Changes, typical for a chronic myeloid leukosis, include: anemia, presence of single miyeloblast and granulocytes at a different stage of a differentiation; in the period of blastny crisis the quantity of blastny cages increases more than by 20%. At a chronic limfoleykoz act as the defining hematologic signs expressed and , existence of limfoblast and Botkin-Gumprekhta's cells.
For the purpose of definition of morphology of a tumoral substratum performance of a sternalny puncture, trepanobiopsiya, biopsy of lymph nodes is shown. In a marrow punktata at chronic myeloid leukemia the quantity of miyelokariotsit at the expense of unripe cages of a granulotsitarny row is increased; in a trepanobioptata replacement of fatty tissue myeloid is defined. At a chronic lymphoid leukosis of a miyelogramm it is characterized by sharp strengthening of a lymphocytic metaplaziya.
Tool researches are applied to assessment of expressiveness of a limfoproliferativny syndrome: Ultrasonography of lymph nodes, spleens, thorax X-ray analysis, limfostsintigrafiya, MSKT of an abdominal cavity and some other.
Treatment and forecast of a chronic leukosis
At an early preclinical stage treatment is inefficient therefore patients are subject to dynamic observation. All-regime actions assume an exception of physical overworks, stresses, insolations, electroprocedures and heattreatment; the good vitaminized nutrition, long walks in the fresh air.
In the developed period of myeloid leukemia chemotherapeutic treatment (, is appointed, hydroxyurea, etc.), at the expressed splenomegaliya is carried out radiation of a spleen. Similar tactics, though does not lead to full treatment, but significantly slows down progressing of a disease and allows to delay approach of blastny crisis. Except medicamentous therapy, at a chronic miyelotsitarny leukosis procedures of a leykaferez are used. In some cases treatment is reached by means of transplantation of marrow.
Upon transition of chronic myeloid leukemia to a terminal stage the high-dose polychemotherapy is appointed. On average after establishment of the diagnosis patients with chronic myeloid leukemia live 3-5 years, in some cases – 10-15 years.
At a chronic limfoleykoz cytostatic therapy (, ), sometimes in a combination to steroid therapy, radiation of lymph nodes, a spleen, skin is also carried out. At significant increase in a spleen the splenektomiya is carried out. Transplantation of stem cells is applied, however its efficiency still demands confirmation. Life expectancy of patients with a chronic lymphoid leukosis can make of 2-3 years (at the heavy, steadily progressing forms) up to 20-25 years (at rather favorable current).