Partial Red Shell Aplasia (PKKA) is a syndrome with a sharp suppression of erythrocyte products, isolated by normochromic anemia and deep reticulocyte.
PcKka first described in 1922 Kaznelson. In the future, a number of cases of this disease were described, while in a significant part of the patients, a tumor of the fork gland (timoma) was revealed.
The subsequent appeared descriptions of the congenital form of the PKK, which manifested itself in the first 2 years of life. Currently, no more than 300 PKKA patients are described and it is shown that this is not one, but several various diseases. In some cases, even with long-term observation, the patient cannot identify the connection of the PKK with any other diseases (idiopathic shape of the PKKA). In other cases, the PKKa is associated with a tumor of the fork gland; Often, this syndrome becomes the first manifestation of any hemoblastosis. Some authors allocate a special (teenage) shape of the PKK with a favorable flow.
Clinical signs
The disease begins gradually. Sick complaints: making up, sharp weakness, fast fatigue, heart pain.
With an objective examination of such patients, the pallor of the skin, as well as visible mucous membranes, is lacking their jaggility. As a rule, the body temperature is within normal limits. In connection with the hemosiderosis, the liver often increases. The spleen is rarely increased.
Painting of blood
In the majority of PKK patients, severe normal anemia with a small amount of reticulocytes is detected; The number of leukocytes is more often normal or even increased, but a number of patients are detected moderate leukopenia, sometimes a neutrophilic shift to the left. The number of platelets is more often normal, much less frequently reduced. ESO is significantly increased.
In the bone marrow, the oppression of the erythroid sprout is often detected at normal content Megakaryocytes and granulocytes, sometimes phagocytosis of erythrocarocytes by macrophages. In the bone marrow, the ratio between the hematopoiet and fat is normal; Characterized structural changes.
Chronic, in some cases it turns out to be achieved by remission, but most therapeutic measures do not lead to the complete normalization of hematological indicators. A number of patients on the background of the PKK are beginning to gradually show signs of hemoblastosis. The rod shift or resembling Pelgar Anomaly is detected, basophilia, eosinophilia appear, sometimes modification. A cytogenetic study at the initial stages of the disease does not reveal changes. In rare cases, as the process progressing, a clone of tumor (leukemic) cells can be revealed. Sometimes there are gradually the signs of a peculiar myeloproliferative disease without pH "- chromosomes. In some cases, erythromyelosis develops, acute undifferentiated leukemia, in which an erythrobist antigen can be detected on the surface of the blasts. A m-gradient of patients with PKK can be detected, which most often included IgG.
Teenage form of PKKa. There is a special form of the disease, detected between the ages of 12 and 22 years. This form also begins gradually, but develops faster than adults. In some patients, it is possible to underk the spleen. Morphological changes are the same as the PKK in adults: pronounced anemia in the absence of reticulocytes and normal number of neutrophils and platelets, the absence or a sharp decrease in the content of erythrocariocyters in the bone marrow.
In contrast to antibodies characteristic of autoimmune hemolytic anemia with incomplete thermal agglutinins, antibodies removed from the surface of erythrocytes of PKKA patients are recorded to all donor erythrocytes, except for papain treated. Antibodies with erythrocytes of patients with autoimmune hemolytic anemia with incomplete thermal agglutinins are recorded both on unchanged donor erythrocytes and on papain treated. The value of these antibodies in the mechanism of development of the PKK is unclear, but they are detected in both adults suffering from PKKA and in teenage and congenital formas of the PKK.
A M-gradient is detected in a large part of the PKK PKK in serum, i.e. there are monoclonal antibodies.
The mechanism for the development of the teenage form is not clear to the end. Many works are devoted to the study of the congenital form of Daieta-blufffan. It is known that this disease is hereditary. In some children with a red sprout reduction, an inhibitor referred to the IGG class was found, but they had another form of the disease - the so-called transient erythroblasting, giving spontaneous remissions. There is evidence that in the syndrome of Daemanda-Blackfuna there are immune lymphocytes that violate erythroid blood formation, but strict evidence is not received.
Daemanda-blukefana syndrome. The disease usually begins in children under 4 months; Pay attention to the sharp pallor of the child. Sometimes, with random blood test, a pronounced anemia is detected. The disease amazes equally often children of both sexes.
As with the anemia, Fanconi, with dyemond-bluer syndrome, there are sometimes changes in large fingers. In addition, some patients have a short neck, as in Sherosezhevsky-Turner syndrome. The increase in liver and spleen is uncharacterically, with the exception of patients who received numerous hemotransphus. In these cases, the geomethium and an increase in the spleen are associated with the hemosiderosis of organs. Frequent symptom - growth delay.
In blood tests - pronounced anemia, reticulocytopic, oppression of a red sprout bone marrow With the normal number of neutrophils and platelets.
Increased content of fetal hemoglobin. Daemondd with co-authors found that the level of fetal hemoglobin in 9 of 12 six-month children ranges from 5 to 25%, while in children of the control group, its content did not exceed 5%.
To identify the antibodies to the erythroblastic antigen in serum in children fails. On the surface of the erythrocytes with the help of an aggregate hemaggluturation sample, antibodies are found, more often than IGA class, less commonly - IgG.
Diagnostics
About the PKK in adults should be thought when, with severe anemia, the reticulous changes are missing or sharply reduced, and the level of platelets and neutrophils is normal or almost normal. There are more often in the bone marrow or almost absent erythrocariocytes in the normal number of neutrophils and megacariocytes, there is no increase in the number of blasts. It should be noted that the reduction of a red sprout is developing not only with a PKKA. A fairly frequent phenomenon is its development under the usual form of autoimmune hemolytic anemia with incomplete thermal agglutinins during a period of severe exacerbation. Since antibodies become much, they destroy not only peripheral erythrocytes, against the antigen of which they are directed, but also erythrocytes, on the surface of which there is also this antigen, but in much smaller quantity. With such forms, in contrast to PKK, the temperature is increased. Helps the study of the specificity of antibodies.
After detection of the PKK, the tumor of the Village gland should be excluded, for this, X-rayly carefully examine the front mediastinum, and the pneumomediastinogram is made in suspected.
Children in the syndrome of Daemond-Blackfast are the same blood changes. Children easily react to treatment with glucocorticosteroids, therefore diagnostic errors are possible, if the content of the reticulocytes is first investigated and sterval puncture is produced after the prednisone appointment.
In these cases, irritation of the red sprout of bone marrow, and not his oppression and increased content Reticulocytes, not reduced. It is necessary to explore the bone marrow and the content of reticulocytes before the prednisian appointment or after some time after its cancellation.
Treatment of PKKE requires a long time and is not always effective, but ensures improvement of more than half of the patients. If the PKKA is a consequence of a tumor of the fork gland, then the removal of the fork gland is necessary, although the operation without additional treatment does not always lead to an improvement in the state. Large doses of prednisolone are effective in teenage form of the PKK, with dyemond-blukefana syndrome and rarely improve the condition of adults. More often prednisone if efficient, then temporarily. In adolescents, good results gives the removal of the spleen, which in itself can lead to an improvement, in adults it is extremely rare. During the removal of the spleen, the doctor prescribes prednisolone to prevent the reduction in the blood of corticosteroids, since the patients take it for a long time before the operation. As the prevention of thrombosis, heparin is used, inhibited in the skin of the abdomen of 5,000 cells 2-3 times a day. In the future, with the same purpose, heparin is replaced by Kurantyl.
In cases where the removal of the spleen does not bring results, cytostatics use. First treated with one drug; specify the best in advance effective drug It is impossible. For the prevention of hemosiderosis, do descell use. In some cases, repeated plasmapheresses are useful.
In patients with a tumor of the fork gland, after removal of the tumor, one of the cytostatic drugs are prescribed.

A sharply occurring disease, accompanied by necrotic processes, hemorrhagic diathesis, a grid form of anemia, is called poleeloftis or an aplasia of bone marrow, what is it and what causes the disease?

The disease is at the heart of the disease, there is a violation of hemopoiesz, that is, the process of education, development, ripening of blood cells - platelets, leukocytes, and erythrocytes. Under the pamilofthylation there is a substitution of blood-forming cells with fatty; Poisoning of chemical agents, strong ionizing radiation may cause an aplasia of bone marrow, what is it, what are the methods of treatment and diagnostics?

Signs of the disease are infectious complications and bleeding caused by a decrease in the concentration of uniform components of blood, while there is no blood regeneration. Easy sharp I. chronic flow Diseases, it can be observed with angina, sepsis, tuberculosis, radiation and cytostatic diseases, poisoning salts of heavy metals, metastasis in bone marrow.

The symptoms of the disease include several syndromes expressed in anemia (lowering the level of erythrocytes), thrombocytopenia (decrease in platelets) and lymphopication (reducing the number of lymphocytes). Patients have a pallor of skin cover, fast fatigue, weakness, with low loading of heartbeat, shortness of breath, impairment of immune reactions due to leukopenia, which is expressed in the emergence of infectious-inflammatory and purulent necrotic processes. Thrombocytopenia provokes development hemorrhagic syndromeexpressed by small and large hemorrhages of different localization, nasal, intestinal, uterine bleeding are not excluded.

It is believed that the cause of the aplasia of the bone marrow can be increased sensitivity of patients to any drugs. Such reactions of the human body are unpredictable, the severity of the disease with the dosage of the drug and the length of therapy is not related, often the development of the aplasia of the bone marrow develops against the background of receiving Levomycetin and other antibiotics.

The physical factors of the development of the aplasia of the bone marrow include ionizing radiation, which is based on a radioactive decay and nuclear transformations, chemical factors are in effect on the body of heavy metals salts, arsenic.

The disease can be provoked by the hepatitis, herpes virus, HIV, systemic red lupus, width syndrome, parvovirus infection, cytomegalovirus.

During pregnancy, the Aplasia is very rare, more often the disease can develop against the background of the reception of some medicines before pregnancy, the drug is required for recovery. It does not exclude the interruption of pregnancy, this makes it possible to use anabolic steroids, in the event of pregnancy, maternal mortality is 10-15%, the fetal death occurs.

If the disease is observed in childhood, we can talk about its hereditary nature, the first signs of the disease in children are manifested in 5-10 years, patients complain about headaches, general weakness, have a tendency to colds.

The diagnostic methods include histological examination, the ipotalobiopyia of the iliac bone is used, the blood tests, feces, urine are prescribed.

Therapeutic methods are consisting of corticosteroids and anabolic hormones, cytostatics, hematopoietic growth factors controlling hemopoiesis, immunosuppressive therapy.

The most progressive methods of treating the disease include the bone marrow transplantation, but the disadvantage of this method is that persistent remission is observed only in 50% of patients.

The most important body in the human body is the bone marrow, which carries out hemopoies, it also enters the immunopower system responsible for the ripening of the cells of the immune system. The main function of the bone marrow is blood formation, it is involved in protective and immunological processes, costhics, mineral, protein, intermediate, fat, carbohydrate exchanges, iron metabolism, ascorbic acid deposit, cholesterol.

When aplasy, the bone marrow function of the body is broken, it leads human body To many adverse consequences, the pancytopenic syndrome occurs. It consists in complete devastation of the bone marrow, this state is extremely dangerous for the life of patients and requires immediate therapy.

Pancitopenia has extensive consequences for the entire body, leading to a lack of oxygen, and also causes problems with immune function. Aplastic anemia or panmyeloftis - other pancytopenia names.

Forms and symptoms of pancytopenia

Pancitolation usually occurs in two forms: idiopathic, the cause of which is not known, but often carries an autoimmune character (means that the body attacks its own fabrics, such as foreign substances) and secondary, caused by environmental factors.

Approximately half of all cases of barcitopenia refers to idiopathic. In addition, viral infections, radiation and chemotherapy, as well as the body's reaction to drugs and the effects of toxins can accelerate the development of pancytopenia.

The form of the disease with defects can be determined as congenital, that is, transmitted by inheritance. Violations in the work of the bone marrow still in the womb of the mother lead to the fact that a child is born with a violation of various systems and internal organs.

Common symptoms of pancytopenia: fatigue, weakness, manifestation of skin defects, such as rashes or non-baking kind of "referring" skin. There may be bleeding from the nose, gums, as well as the bleeding of internal organs.

Additional symptoms: pallor, frequent viral infections, pale leather, tachycardia, biting, bruises on the surface of the skin, weakness.

Causes of pancytopenia and risk factors

Pancitopenia can be caused by hereditary causes (gene mutations), drugs or influence of environmental factors such as radiation or arsenic. Approximately half of the cases of barcitopenia, the disease has an idiopathic shape, and it cannot be accurately determined. An autoimmune violation can be based on, in which the body destroys its own cells and fabrics as foreign substances or impurities in the environment. In very rare cases, pregnancy can lead to this state.

Definition of reasons is very important for the doctor, since this will depend on this method of treatment. For example, pancytopenia caused by an environmental factor can be adjusted by removing the poisoning substance and the normalization of living conditions.

Risk Risk Factors

A number of factors increase the risk of racitopenia, but not all people living in conditions of increased risk or exposed to factors are ill.

Substances and factors most often causing pancytopenia:

• interaction with environmental toxins, such as benzene or arsenic;
• family history of blood diseases;
• lupus or some other autoimmune diseases;
• pregnancy (very rarely);
• radiation therapy;
• antibiotics, immunosuppressants.
• chemotherapeutic drugs;
• radiation;
• viral infections.

Treatment of pancytopenia

Congenital shape of the pancytopenia is treated according to the scheme that the doctor defines in each case. With mild or moderate manifestations, treatment may not be obligatory, however, with more serious forms of the disease, blood transfusion is relevant (it helps to restore the balance of blood taurus).

However, over time, blood transfusion loses its effectiveness. A more radical treatment is a bone marrow transplant or stem cell therapy. These procedures restore the ability of bone marrow to produce blood cells. Such procedures are usually effective for young patients, but the elderly can also be useful to use drugs that stimulate the activity of bone marrow.

In cases associated with environmental factors, pancytopenia can only be defeated by the elimination of the external factor - any toxin or poisoning substance.

If the main cause of the disease is the attack of the immune system to the bone marrow, immunosuppressants are prescribed:

Stimulating bone marrow preparations:

• epoetin Alpha (Epogen, Correction);
• philgrantim (neapogen);
• Pegfilgrastim (neulasta);
• Sargramostim (Leukine, Prokine).

What are the potential complications caused by pancytopenia?

In the absence of treatment of pancytopenia, it may lead to a life-threatening bleeding and development of infections. Complications are more often in older patients.

Bone marrow hypoplasia: symptoms and treatment

Gipoplasia, or bone marrow deficiency is a group of disorders acquired or hereditary. They imply the disorders of the hematopoietic system, including deficiency of platelets, red blood cells, myeloid cells.

Inherited forms of bone marrow deficiency is: Fanconi anemia, congenital discratosis, Daimond-Flephane anemia and other genetic diseases. The most common cause of acquired bone marrow hypoplasia is aplastic anemia. Diseases that appear as a result of acquired bone marrow hypoplasia: myelodsplastic syndrome, paroxysmal night hemoglobinuria and granular lympholecosis.

Patients with bone marrow failure have a small amount of blood compared to healthy patients. Reducing the number of platelets makes patients predisposed to spontaneous bleeding from cuts and skin injuries, and also increases the bleeding of mucous membranes. The disease develops slowly, for several months.

Healthy bone marrow hypoplasia is usually diagnosed in young patients, as well as patients over 60 years old.

Any of these diseases can be alarmed about bone marrow hypoplasia: hematological cytopenia, inexplicable macrocytosis, myelodsplastic syndrome or acute myelolomicosis, flat-mellular cancer.

For the treatment of bone marrow hypoplasia, preparations are used, which are usually prescribed for anemia, but they are rarely effective. Usually they are used as auxiliary means, and the main treatment is carried out using:

• corticosteroids;
• blood transfusion (must be carried out at intervals, since the procedure is addictive and is not so useful for the body).

Some cases of this disease are effectively treated with splenectomy.

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The reason for the development of bone marrow hypoplasia can be increased sensitivity to the drug drug. The reaction of this type is unpredictable and does not have the relationship between the dose of the drug and the duration of the reception. Most often, the hypoplasia is caused by Lewomycin, sulfanimamides, tetracycline, antihistamines, barbiturates, etc. The hypoplasia can cause viral infections ( viral hepatitis B, Epstein-Barr virus, herpes virus, cytomegalovirus).

Treatment is a rather complicated task. Basic and only treatment is bone marrow transplantation from a compatible donor.

In the absence of the possibility of choosing a donor the paraliative therapy will cause. The method of choice is immunosuppressive therapy, it is comparable to transplantation of stem cells, but with a smaller number of fatal complications. Immunosuppressive therapy includes anti-lumphocytic or antimonocytic immunoglobulin, cyclosporin A and corticosteroid hormonal drugs. As immunosuppressive therapy, it is sometimes considered to be a spynectomy (removal of the spleen). All patients with hypoplasia (aplasia) of bone marrow need transfusion therapy for erythrocytes and / or thrombocytees. In addition, antibacterial and microwatical therapy is carried out in order to prevent or treat infectious complications.

So everything is not easy. Treatment must be coordinated in hematologist

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Bone marrow hypoplasia

Bone marrow hypoplasia (Hypoplasia Medullae Ossium; Greek. Hypo- - under, decrease, insufficiency + plasis - education, formation) - the state of the bone marrow, in which the resignation of the myeloid bone marrow tissue with a fatty tissue and, as a result, progresses the decrease in the intensity of leoko- erythro and thrombocytopower; It is observed in chronic infections, hypoplastic anemia, metastatic and systemic lesions of the bone marrow.

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Question # 6 - What is bone marrow hypoplasia?

Artemieva Veronica from Nizhny Tagila asks:

What is bone marrow hypoplasia, and what symptoms is this disease accompanied?

Expert answer:

The bone marrow hypoplasia is a state at which myeloid tissue is replaced by fatty. The very concept of "hypoplasia" translated means insufficiency of the formation. In case of insufficient formation of myeloid tissue, the function of the red bone marrow is disturbed, as a result of which the production of blood cells - leukocytes, erythrocytes, platelets is significantly reduced. The insufficiency of the bone marrow is one of the types of pancytopenia.

Causes of development

There are two forms of illness:

The cause of the development of hereditary forms is the following pathologies:

• anemia of FanConi;
• congenital discratosis;
• daimond-Blefen anemia;
• other genetic diseases.

Insufficiency of blood cells can act as an independent disease in aplastic anemia or develop against the background of the following diseases:

• liver cirrhosis;
• chronic hepatitis;
• malignant neoplasms;
• different autoimmune disorders.

Manifestations of disease

In the body of patients with people, the blood volume is much lower than in healthy. As a result of reducing the number of platelets, patients have spontaneous bleeding. Danger can represent any cuts, injuries leading to significant blood loss. Bloodstuffs are subject to mucous membranes and internal organs.

The insufficiency of the production of leukocytes leads to a decrease in immunity that contributes to the occurrence of frequent infectious diseases.

Principles of treatment

The treatment of this pathology is engaged in a hematologist. The choice of the method of therapy depends on the cause of the occurrence of the disease. Aplastic anemia is possible to eliminate only by transplanting bone marrow. If it fails to find a suitable donor, the patient shows the reception of drugs depressing immune system (Cyclosporine a). Immunosuppressive therapy allows us to succeed only with non-heavy forms of illness.

Everybody without exception, patients produce intravenous injection of platelet and erythrocyte mass. In order to prevent the development of infectious and fungal lesions, the treatment of antibacterial and antifungal drugs is prescribed to the patient.

One of the causes of insufficient blood cell content is to increase the activity of the spleen - hyperplanism. Therefore, patients can be performed by splectomy - operation, during which the spleen removes.

Video: What is bone marrow transplantation

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Aplasia bone marrow

The aplasia of the bone marrow (or aplasia of the blood formation) is the syndromes of bone marrow deficiency, which include a group of disorders in which the function of the blood formation is sharply suppressed. The consequence of this violation is the development of pancytopenia (there is a shortage of all blood cells: leukocytes, erythrocytes, as well as platelets). Deep pancytopenia is a life-threatening condition.

Code of the ICD-10

Epidemiology

The aplasia of the bone marrow occurs in people with a frequency of 2.0 / people annually. This indicator varies depending on the country, therefore, there may be a scatter within 0.6-3.0 + / people for annually.

Causes of bone marrow aplas

Among the causes of the aplasia of the bone marrow are distinguished by such:

• Chemo and radiation therapy.
• Autoimmune violations.
• Environmentally harmful working conditions.
• Various viral infections.
• Contact with herbicides and insecticides.
• Some medicines, for example, drugs treated rheumatoid arthritis, or antibiotics.
• Night hemoglobinuria.
• Hemolytic anemia.
• Diseases of connective tissue.
• Pregnancy - bone marrow is affected due to the perverted reaction of the immune system.

Risk factors

Among the risk factors of the aplasia of the bone marrow are allocated as described below.

• chemical compounds: cytostatics - they contribute to the cessation of cell division, they are usually used for tumor therapy. A certain dosage of such drugs may damage the bone marrow, violating the formation of blood cells; Immunosuppressants - suppress the body's immune system, they are used when there is an excessive activation of immunity at which their own healthy fabric is damaged. If we cancel them, the blood formation is often restored;
• substances that affect the organism if the patient has individual hypersensitivity to them. These are antibiotics (antibacterial drugs), gasoline, mercury, various dyes, chloramphenicol, as well as gold preparations. Such substances are able to cause both reversible, and irreversible destruction of the bone marrow function. They can get into the body through the skin, when breathing with an aerosol manner, orally - along with water and food;
• irradiation with ionic particles (radiation) - for example, if the safety regulations at nuclear power plants or medical institutions are violated, where tumors are treated with radiation therapy;
• viral infections - such as influenza, hepatitis virus, etc.

Pathogenesis

The pathogenesis of the aplasia of the bone marrow has not yet been studied completely. Nowadays, several different mechanisms for its development are considered:

• The bone marrow is amazed through a polypotent stem cell;
• The hematopoietic process is suppressed due to the impact on it of humoral or cellular immune mechanisms;
• Microeration components begin to function incorrectly;
• The development of the deficit of factors that contribute to the hematopoietic process.
• Mutations in genes, causing hereditary syndromes of bone marrow deficiency.

With this disease, the content of components (this is vitamin B12, iron, as well as protoporphine), which directly participate in blood formation, does not decrease, but at the same time, the blood-made cloth cannot use them.

Symptoms of aplasia of bone marrow

The aplasia of bone marrow manifests itself depending on which cell element of blood was amazed:

• If there is a decrease in the level of erythrocytes, shortness of breath appears and total weakness and other symptoms of anemia;
• If the leukocyte level decreases, fever occurs and the susceptibility of the body is increasing to infections;
• If the level of platelets is reduced, the tendency to the development of hemorrhagic syndrome, the occurrence of petechs, as well as to bleeding.

For partaman Red Bone Camopy Aplay There is a sharp decrease in erythrocyte production, deep reticulocytopenia, as well as isolated normochromic anemia.

There is a congenital and acquired forms of this disease. The second manifests itself under the guise of acquired primary erythrobredophtis, as well as the syndrome that occurs with other diseases (it can be lung cancer, hepatitis, leukemia, infectious mononucleosis or pneumonia, as well as sickle-cell anemia, vapotitis or ulcerative colitis, etc.).

Complications and consequences

Among the complications of the aplasia of the bone marrow:

• Anemic Coma, in which the loss of consciousness occurs, the development of a comatose state. There is no reaction to any external stimuli, since oxygen does not enter the brain in the necessary quantities - this is due to the fact that the level of erythrocytes in the blood is quickly and significantly reduced;
• A variety of bleeding begin (hemorrhagic complications). The worst option in this case is hemorrhagic stroke (some of the brain is impregnated with blood and as a result of this dies);
• Infections - microorganisms (a variety of fungi, bacteria or viruses) become the cause of infectious diseases;
• Violation functional state Some internal organs (such as kidneys or heart), especially with the accomplishing chronic pathology.

Diagnosis of bone marrow aplasa

When diagnosing an aplasia of the bone marrow, the history of the disease is carried out, as well as patient complaints: how long the symptoms of the disease appeared, and with which the patient binds their appearance.

• The presence of a patient with accompanying chronic diseases.
• The presence of hereditary diseases.
• Does the patient have bad habits.
• Refine whether the recently long-term use of any drugs was carried out.
• The presence of patient tumors.
• Whether contact with various toxic elements was.
• It turned out to be the effect of radiation irradiation or other radiation factors.

After that, a physical inspection is carried out. The skin color is determined (in the aplasia of the bone marrow there is a pallor), the pulse frequency is determined (most often it is rapidly) and the blood pressure indicators (it is reduced). The mucous membranes and skin cover are examined for the presence of hemorrhages and purulent bubbles, etc.

Analyzes

In the process of diagnosing the disease, some laboratory studies are carried out.

A blood test is performed - if the patient has an aplasia of the bone marrow, a decrease in the level of hemoglobin, as well as the number of erythrocytes, will be revealed. The color indicator of the blood flow is in normal. The number of platelets with leukocytes decreases, and in addition, the correct ratio of leukocytes is disturbed, because the content of granulocytes is reduced.

A urine analysis is also carried out to determine the presence of erythrocytes in the urine - this is a sign of hemorrhagic syndrome, or the presence of leukocytes and microorganisms, which is the symptom of development in the body of infectious complications.

A biochemical blood test is also carried out. Thanks to it, the indicators of glucose, cholesterol, uric acid (in order to identify the accompanying defeat of any organs), creatinine, as well as electrolytes (sodium, potassium and calcium).

Instrumental diagnostics

When instrumental diagnostics, the following procedures are carried out.

In order to survey the bone marrow, puncture is performed (piercing at which the internal contents) of any bone is extracted, it is usually such a chest or a hip bone. With the help of a microscopic examination, the replacement of the hematopoietic fabric on the scarsing or fat is determined.

Trepanobiopia, in which the bone marrow is examined, as well as its ratio with nearby tissues. During this procedure, a special apparatus is used, which is called Trepan - with its help from the iliac bone, the bone marrow bar is taken together with the perception, as well as the bone.

Electrocardiography that allows you to identify problems with nutrition of the heart muscle, heart rhythm.

What tests are necessary?

Differential diagnosis

Differential diagnosis is carried out with such diseases:

Who to contact?

Treatment of aplasia of bone marrow

Eliminate the disease with etiotropic treatment (affecting its cause) is almost impossible. Can help eliminate provoking factor (for example, cancellation of the received medicinal preparation, departure from the radiation zone, etc.), but in this case only the speed of the death of the bone marrow is reduced, but it is not possible to restore stable blood formation in this way.

Immunosuppressive treatment is carried out if there is no transplantation (no suggestive donor patient). In this case, drugs are used from cyclosporine groups or either anti-fluocytic globulin. Sometimes they are used together.

The use of GM CSF (drugs that stimulate the production of leukocytes). This treatment is used if the number of leukocytes drops to less than 2x109 g / l. Also in this case, corticosteroid drugs can be used.

Anabolic steroids are used, which stimulate the formation of protein.

In the process of treating the aplasia of bone marrow, such methods are applied:

Mixed erythrocytes are transfused (these are donor erythrocytes that exempt from proteins) - this method reduces the severity and number of negative reactions to the transfusion procedure. Do such a transfusion only if there is a threat to the patient's life. These are the following states:

• the patient flows into anemic to whom;
• anemia of a heavy degree of gravity (in this case, the hemoglobin level indicator drops below 70 g / l).

The transfusion of donor platelets is carried out if the patient has bleeding and a pronounced decrease in the number of platelets.

The hemostatic therapy is carried out depending on the area in which bleeding began.

In the event of infectious complications, therapy methods are carried out:

• antibacterial treatment. It is performed after the strokes from the nasopharynk are taken, as well as the urine and blood for sowing, to determine which microorganism caused the occurrence of infection, as well as reveal its sensitivity to antibiotics;
• systemic antifungal treatment is performed;
• local treatment with antiseptic areas that can become an entrance gate of infection (these are those places through which bacteria, fungi or viruses fall into the body). Under such procedures, the mouth rinse is usually meant using different drugs.

Medicine

In aplasia of the bone marrow is necessarily used medicia treatment. Most often, drugs belonging to 3 drug groups are used: these are cytostatic (6-mercaptopuril, cyclophosphane, methotrexate, cyclosporin A, as well as Imuran), immunosuppressants (dexamethasone, as well as methylprednisolone) and antibiotics (macrolides, cephalosporins, chlorineolone, and azalida ). Sometimes medications can be used, corrective disorders of intestinal microflora and control problems, enzyme drugs, etc.

Methylprednisolone is prescribed inside. When transplanting organs - in the dosage of not more than 0.007 g / day.

The side effects of the drug: Water, as well as sodium can linger in the body, increases blood pressure, the loss of potassium, osteoporosis, muscular weakness, medicinal gastritis can be observed; may decrease to various infections; Suppression of adrenal operations, some mental disordersproblems with menstrual cycle.

The medicine is contraindicated with a heavy stage hypertensive disease; at 3 stages of insufficiency of blood circulation, and besides this, during pregnancy and acute endocarditance, as well as jade, various psychosis, osteoporosis, ulcers of a 12 republic or stomach; after recent operation; with the active stage of tuberculosis, syphilis; People of older age, as well as children under 12 years old.

Methylprednisolone is prescribed with caution in the presence of diabetes mellitus, only if there are absolute indications or for treatment in patients with insulin resistance, with high anti-zulin antibodies. With tuberculosis or infectious diseases, it is possible to use a medicine, only combining it with antibiotics or drugs treated with tuberculosis.

Imuran - on the first day it is allowed to apply a dose of not more than 5 mg per 1 kg of human weight per day (it is necessary to use 2-3 receptions), but the dosage as a whole depends on the immunosuppression mode. The size of the supporting dose is 1-4 mg / kg of weight per day. It is established depending on the tolerance of the patient's body and its clinical condition. Studies indicate that treatment with the use of imurana needs to be carried out by a long course, even using small doses.

In case of overdose, ulcers may appear in the throat, bleeding and bruises, as well as infections. Such signs are more characteristic of chronic overdose.

Side effects - After the bone marrow transplantation in combination with other immunosuppressors in patients, bacterial, fungal or viral infections are often observed in patients. Among others adverse Reactions - arrhythmia, signs of meningism, headaches, lip damage and oral cavity, paresthesia, etc.

Cyclosporine A is used intravenously - the daily dose is divided into 2 receptions and is introduced in 2-6 hours. For the initial daily dose, 3-5 mg / kg is sufficient. Intravenous application It is optimal in the process of treating patients who performed bone marrow transplantation. Before transplanting (4-12 hours. Over the operation), the patient is given inside the dosage / kg, and then the next 1-2 weeks apply the same daily dose. Later, the dose is reduced to the usual supporting (approximately 2-6 mg / kg).

The symptoms of overdose are drowsiness, severe vomiting, tachycardia, headaches, the development of heavy renal failure.

When receiving cyclosporin, it is necessary to follow the following precautions. Therapy should be carried out in hospital doctors who have extensive experience in the treatment of patients with immunosuppressants. It should be remembered that due to the reception of cyclosporine, the predisposition to the development of malignant lymphoproliferative tumors increases. That is why it is necessary to decide before the start of reception, whether the positive effect of his treatment has just conjugate risks. During pregnancy, the drug is allowed to use only due to strict indications. Since there is a risk of anaphyactoid reactions as a result of intravenous reception, it is necessary to make antihistamines to take antihistamines, as well as to maximally translate the patient to the oral method for administering the medication.

Vitamins

If the patient has bleeding, in addition to hemotherapy, a 10% calcium chloride solution should be taken (inside), as well as vitamin K (per day). In addition, ascorbic acid is prescribed in large quantities (0.5-1 g / day) and vitamin P (in the dosage of 0.15-0.3 g / day). Recommended to accept foliic acid In large doses (maximum 200 mg / day), as well as vitamin B6, preferably in the form of injections (50 mg of pyridoxine daily).

Physiotherapeutic treatment

To activate the operation of the bone marrow, physiotherapeutic treatment is applied - diathermy of tubular bones in the field of the heads or sternum. The procedure must be carried out every day for 20 minutes. It should be noted that this option is possible only if there is no pronounced bleeding.

Operational treatment

The bone marrow transplant is carried out in the event of a serious stage of Aplasia. The effectiveness of such an operation increases if the patient is at a young age, and he also performed a small amount of transfusions of the elements of donor blood (no more than 10).

With such treatment, it is removed from the bone marrow donor and its further transplantation of the recipient. Before the suspension of stem cells is introduced, they are treated with cytostatics.

After transplantation, the patient has a long-term course of immunosuppressive treatment, which is necessary to prevent possible transplant rejection by the body, as well as preventing other negative immune reactions.

Prevention

Primary preventive measures Regarding aplasia of the bone marrow is as follows: it is necessary to prevent the impact on the body of external negative factors. To do this, followed safety equipment in the process of working with dyes or objects that may be sources of ionizing radiation, as well as monitor the process of use of drugs.

Secondary prophylaxis, which is necessary to prevent a possible deterioration in a person in a person with an extended illness or to prevent recurrence, is such measures:

• Dispensary accounting. The observation must continue, even if there are signs of the patient's recovery;
• Perennial supporting drug therapy.

Forecast

Bone marrow Aplasia usually has an unfavorable forecast - if timely treatment is not carried out, the patient dies in 90% of cases.

Thanks to the transplantation of donor bone marrow, 9 out of 10 patients can live more than 5 years. Therefore, this method is considered the most effective method of treatment.

Sometimes it is not possible to perform a transplant possible, but modern drug therapy is also able to give a result. About half of patients thanks for it can live for more than 5 years. But at the same time, in most cases, patients survive, which are no longer over 40 years old.

Medical Expert Editor

Portnov Alexey Alexandrovich

Education: Kiev National Medical University. A.A. Bogomolets, specialty - "Therapeutic business"

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Simultaneously with signs of violation of various links intracellular metabolism There is a decrease in the number of breeding cells of white and red sprouts of the bone marrow. Interphase death and violation of the method of dividing the cells of myeloid series is occurring. Moreover, it is essential that the external irradiation acts mainly on premotional, and the defeat of the incorporated radioactive substances to postmitic phases of the cell cycle [Husar Yu. P., Syoshovar Yu. A., 1977].

By the end of the first day after irradiation, the number hooping cells sharply decreases. At the same time draws attention to the reduction of red sprout cells. In the bone marrow points, only polychromatophilic and oxyphibal normoblasts are found and there are no passitroblasts, basophilous forms and dividing cells. The numbers of myelocarocytes decrease almost twice as much as the number of immature neutrophilic series (up to 25%) and lymphocytes (up to 60%) are dramatically reduced.

At the same time, reticular cells and other histiocyte elements contained in more quantity than usual. Simultaneously with the collapse of the hematopoietic cells in the bone marrow, macrophages are detected with signs of phagocytosis of cellular daddy.

The main cause of Aplasia hematopoietic fabric with acute radiation disease is the death of polypotent due to direct action Ionizing radiation. The death of blood cells of early generations occurs in an interfass due to necrobiosis and pathological division. When irradiated at a dose of 100, about 63% of stem cells die, and when irradiated at a dose of 1000, they are glad to decrease by 1000 times [G. P. G., 1970].

A sufficient number appeared in the literature information On chromosomal aberrations of bone marrow cells in people randomly subjected to external radiation in massive doses. It is established that the largest number of aberrations is observed after 22-48 hours after irradiation, when the aberration of chromosomal type is dominated. The number of aberrant cells increases in accordance with the increase in the dose of radiation [heels "E. K., Butler L. I., 1968].
On this basis, the criteria of biological dosimetry [Vorobiev A.V. and DR, 1973, 1975].

Through 3 days of foci bleelms It is detected only in the form of individual islands consisting of a few ripe shapes of granulocytes with hypersegmented, picnotic or disintegrating nuclei, few cells of the red row and single disintegrating megacariocytes and a large number of plasma cells. In the future, the amount of myeloid tissue decreases even more. At the height of the disease, the bone marrow is an edema fat fabric, in the stroma of which separate cells or small islands of cell elements are visible.

Minor areas of bone marrow fabric Save near bone beams. The main mass of cells during this period is plasma cells, undifferentiated reticular elements and individual hemocytoblasts. Giant ugly form neutrophilic leukocytes appear with increased DNA content in large, hypersegmented nuclei. According to the observations of D.I. Goldberg et al. (1974), after irradiation in massive doses, the population of radio-resistant reticular cells can be transformed into giant hemogisticoblasts that are capable of differentiate into the cells of myeloid series. This is confirmed by the presence in some of these cells of peroxidase and phospholipnds, characteristic, as known, myeloid cells. At the same time, such gigantic neutrophils are characterized by low peroxidase alkaline phosphatase activity, low content Glycogen, which indicates the inability to provide their specific functions.