Agranulotsitoz – a kliniko-hematologic syndrome which cornerstone sharp reduction or lack of neytrofilny granulocytes among cellular elements of peripheral blood is. Agranulotsitoz is followed by development of infectious processes, quinsies, ulcer stomatitis, pneumonia, hemorrhagic manifestations. From complications sepsis, hepatitis are frequent, mediastinit, peritonitis. Paramount value for diagnostics of an agranulotsitoz has a research of a gemogramma, punktat of marrow, detection of antineytrofilny antibodies. Treatment is directed to elimination of the reasons which caused , the prevention of complications and restoration of blood formation.
Agranulotsitoz – the change of a picture of peripheral blood which is developing at a number of independent diseases and characterized by decrease in quantity or disappearance of granulocytes. In hematology under agranulotsitozy reduction of quantity of granulocytes in blood less than 0,75Õ109/l is meant or 1Õ109/l is lower than total number of leukocytes. Congenital meets extremely seldom; the acquired state is diagnosed with a frequency of 1 a case on 1200 people. Women suffer agranulotsitozy 2-3 times more often than men; usually the syndrome comes to light aged 40 years are more senior. Now in connection with wide use in medical practice of cytotoxic therapy, and also emergence of a large number of new pharmacological means the frequency of cases of an agranulotsitoz considerably increased.
Classification of an agranulotsitoz
First of all, agranulotsitoza are subdivided on congenital and acquired. The last can be an independent pathological condition or one of manifestations of other syndrome. On the leading pathogenetic factor distinguish miyelotoksichesky, immune gaptenovy and autoimmune . Also allocate an idiopathic (genuinny) form with an unspecified etiology.
On features of a clinical current differentiate sharp and recidivous (chronic) agranulotsitoza. Weight of a current of an agranulotsitoz depends on quantity of granulocytes in blood and can be easy (at the level of granulocytes 1,0-0,5Õ109/l), average (at level less than 0,5Õ109/l) or heavy (at total absence of granulocytes in blood).
Role of granulocytes in an organism
Granulocytes are called leukocytes in which cytoplasm when coloring specific granularity (granules) is defined. Granulocytes are developed in marrow therefore treat cages of a myeloid row. They make the most numerous group of leukocytes. Depending on features of coloring of granules these cages are subdivided into neutrophils, eosinophils and basophiles – they differ on the functions in an organism.
About 50-75% of all white blood cells fall to the share of neytrofilny granulocytes. Among them distinguish mature segmentoyaderny (it is normal of 45-70%) and unripe stab neutrophils (it is normal of 1-6%). The state which is characterized by increase in maintenance of neutrophils carries the name of a neytrofiliya; in case of reduction in the amount of neutrophils speak about a neytropeniye (granulotsitopeniye), and in case of absence – about an agranulotsitoza. In an organism neytrofilny granulocytes carry out a role of the main protective factor from infections (mainly, microbic and fungal). At introduction of the infectious agent neutrophils migrate through a wall of capillaries and direct in fabric to the infection center, fagotsitirut and destroy bacteria by the enzymes, actively forming the local inflammatory answer. At an agranulotsitoza reaction of an organism to introduction of the infectious activator turns out inefficient what can be followed by development of fatal septic complications.
Reasons of an agranulotsitoz
Miyelotoksichesky arises owing to suppression of production of cages predecessors of a miyelopoez in marrow. At the same time in blood decrease in level of lymphocytes, retikulotsit, platelets is noted. This type of an agranulotsitoz can develop at impact on an organism of ionizing radiation, cytostatic medicines and other pharmacological means (a levomitsetin, streptomycin, gentamycin, penicillin, colchicine, aminazine), etc.
Immune it is connected with education in an organism of antibodies which action is turned against own leukocytes. Emergence of a gaptenovy immune agranulotsitoz provokes reception of the sulfanylamides NPVS-derivative of a pirazolon (an amidopirin, analginum, aspirin, a butadion), medicines for therapy of tuberculosis, diabetes, helminthoses which act as gapten. They are capable to form complex connections with proteins of blood or covers of leukocytes, becoming anti-genes in relation to which the organism begins to produce antibodies. The last are fixed on a surface of white blood cells, causing their death.
The pathological reaction of immune system which is followed by formation of antineytrofilny antibodies is the cornerstone of an autoimmune agranulotsitoz. Such kind of an agranulotsitoz meets at an autoimmune tireoidit, rheumatoid arthritis, system red a wolf cub and other collagenases. Agranulotsitoz developing at some infectious diseases (flu, an infectious mononukleoz, malaria, yellow fever, a typhoid, viral hepatitis, poliomyelitis, etc.) also has immune character. The expressed neytropeniya can signal about a chronic limfoleykoz, aplastic anemia, Felti's syndrome, and also to proceed in parallel with thrombocytopenia or hemolytic anemia. Congenital is a consequence of genetic disorders.
The pathological reactions accompanying a current of an agranulotsitoz are in most cases presented by ulcer and necrotic changes of skin, mucous membrane of an oral cavity and throat, is more rare - a conjunctival cavity, a throat, a stomach. Necrotic ulcers can arise in mucous intestines, causing perforation of an intestinal wall, development of intestinal bleedings; in a wall of a bladder and vagina. At microscopy of sites of a necrosis lack of neytrofilny granulocytes is found.
Symptoms of an agranulotsitoz
The clinic of an immune agranulotsitoz usually develops sharply, unlike miyelotoksichesky and autoimmune options at which pathological symptoms arise and progress gradually. To early to manifest manifestations of an agranulotsitoz fever (39-40 °C), sharp weakness, pallor, perspiration, artralgiya belong. Processes of a mucous membrane of a mouth and throat (gingivita, stomatitises, pharyngitises, quinsies), a nekrotization of a uvula, soft and hard palate are characteristic ulcer and necrotic. These changes are followed by a salivation, a sore throat, a dysphagy, a spasm of chewing muscles. Regionarny lymphadenitis, moderate increase in a liver and spleen is noted.
For a miyelotoksichesky agranulotsitoz typically emergence of moderately expressed hemorrhagic syndrome which is shown bleeding of gums, nasal bleedings, formation of bruises and hematomas, a gematuriya. At damage of intestines the necrotic enteropatiya as which manifestations serve skhvatkoobrazny belly-aches, diarrhea, an abdominal distension develops. At a severe form complications in the form of a perforation of intestines, peritonitis are possible.
At an agranulotsitoza patients can have hemorrhagic pneumonia which is complicated by abscesses and gangrene of a lung. At the same time fizikalny and radiological data happen extremely poor. From among the most frequent complications perforation of a soft palate, sepsis are possible, mediastinit, an acute hepatitis.
Diagnostics of an agranulotsitoz
The group of potential risk on development of an agranulotsitoz is made by the patients who had a serious infectious disease, receiving radiation, cytotoxic or other medicinal therapy, suffering from collagenases. From clinical data diagnostic value represents a combination of a hyperthermia, ulcer and necrotic defeats of visible mucous and hemorrhagic manifestations.
The most important for confirmation of an agranulotsitoz is the research of the general blood test and a puncture of marrow. The picture of peripheral blood is characterized by a leykopeniya (1-2Õ109/l), a granulotsitopeniya (less than 0,75Õ109/l) or agranulotsitozy, moderate anemia, at heavy degrees – thrombocytopenia. At a research of a miyelogramma reduction of quantity of miyelokariotsit, decrease in number and violation of maturing of cages of a neytrofilny sprout, existence of a large number of plasmatic cages and megakariotsit comes to light. Definition of antineytrofilny antibodies is made for confirmation of autoimmune character of an agranulotsitoz.
Carrying out a X-ray analysis of lungs, repeated blood tests on sterility, a research of biochemical blood test, consultation of the stomatologist and otolaryngologist is shown to all patients with agranulotsitozy. It is necessary to differentiate from a sharp leukosis, hypoplastic anemia. The HIV status exception is also necessary.
Treatment and prevention of an agranulotsitoz
Patients with verified agranulotsitozy have to be hospitalized in office of hematology. Patients are located in chamber insulator with aseptic conditions where regular quartz treatment is carried out, visit is limited, the medical personnel work only in hats, masks and boot covers. These measures are directed to the prevention of infectious complications. In case of development of a necrotic enteropatiya a transfer of the patient on parenteral food is made. Careful care of an oral cavity is necessary for patients with agranulotsitozy (frequent rinsings of a mouth antiseptic solutions, greasing of mucous membranes).
Therapy of an agranulotsitoz begins with elimination of an etiologichesky factor (cancellation of miyelotoksichesky medicines and chemicals etc.). For prevention of a purulent infection not absorbed antibiotics, antifungal medicines are appointed. Intravenous administration of immunoglobulin and anti-staphylococcal plasma, a transfusion of leykotsitarny weight is shown, at a hemorrhagic syndrome - trombotsitarny weight. At immune and autoimmune character of an agranulotsitoz glyukortikoida in high doses are appointed. In the presence the plasma exchange is carried out to blood of the CEC and antibodies. In complex treatment of an agranulotsitoz stimulators of a leykopoez are used.
Prevention of an agranulotsitoz, mainly, consists in carrying out careful hematologic control during a course of treatment miyelotoksichesky medicines, an exception of repeated drug intake, earlier caused in the sick phenomenon of an immune agranulotsitoz. The adverse forecast is observed at development of heavy septic complications, repeated development of gaptenovy agranulotsitoz