Pancytopenia is a hematological concept, which means a decrease in the level of all types of peripheral blood cells. In connection with a sharp decrease in the number of all types of blood cells in the clinical picture of pancytopenia, three main syndrome: hemorrhagic, infectious and anemic. These three syndromes are the "calling card" of pancytopenia.
People of any age, race and sex are prone to pancytopenia. The causes of this condition with the same success are both congenital and acquired diseases. In addition, pancytopenia is a constant companion of chemotherapy and cytostatic drugs.
What is pancytopenia
Pancytopenia is a condition of insufficiency of all types of blood cells. The development of such a state is possible if the bone marrow is not working properly.
A cell-mother for all blood elements is a polypotent stem cell. It is one for all blood cells and there is no difference what kind of shaped element it will come from. The repository of pluripotent stem cells is the red bone marrow, in which the maturation of blood cells occurs.
The polypotent stem cell forms a self-sustaining cell population and has the ability to actively divide. The first three classes of cells form a class of morphologically unrecognizable cells (all cells of these classes are identical), but after a number of divisions the cells acquire small differences, the myeloid and lymphoid cell sprouts are isolated. Progenitors (blast cells) of leukocytes, platelets and erythrocytes are formed from the myeloid sprout, while the progenitor cells (blasts) of T-lymphocytes, B-lymphocytes and NK-killers that play an important role in the defense mechanisms of the immune system mature out of the lymphocyte germ . Blastnye cells of different shoots do not have bright differences. Further, the blasts pass through the stages of division, differentiation, gradually maturing to full blood cells. Mature cells leave the red bone marrow and begin to circulate in the general blood stream.
In the event of a deterioration in the maturation of the progenitor cells of one of the germs, blast cells will emerge into the peripheral blood, which can be determined by a clinical blood test. In addition to blasts, there will be a decrease in the number of blood cells that must mature from the affected shoot.
If the lesion occurs at earlier levels, then in the blood there will be a total decrease in the number of all types of blood cells - pancytopenia. This condition is possible with aplasia or hypoplasia of the red bone marrow.
Causes of pancytopenia
The causes of pancytopenia have not been fully investigated, therefore this condition remains in the idiopathic category, i.e. with an unclear reason.
Types of pancytopenia are conventionally divided into idiopathic pancytopenia and pancytopenia with established cause.
In addition, there are also types of pancytopenia, such as acquired and congenital.
The causes suppressing the function of the bone marrow can be toxins, ionizing radiation, chemotherapy, radiation therapy, viral agents, intake of certain groups of drugs, autoimmune diseases, environmental disasters, genetic defects of the hematopoiesis system.
The identification of the causes of the syndrome of pancytopenia is very important and is a prerequisite for the successful treatment of this condition.
Pancytopenia is more common in chemotherapy blocks during the treatment of malignant neoplasms. Chemotherapy causes the death of blast cells of both myeloid and lymphoid bone marrow sprouts. Incidentally, with leukemia this is not a side effect, but the goal of treatment, as diseased cellular elements as well as normal blast cells die. This condition of the red bone marrow is called desolation.
There is a disease not associated with a toxic effect on the bone marrow, but is the cause of pancytopenia. This ailment is called osteopetrosis. With this disease, bone marrow transplantation occurs with bone septa. As a result, too little space remains for the hematopoietic tissue of the red brain, and hematopoiesis becomes ineffective. Peripheral blood shows pancytopenia and anemia .
As a congenital cause of the described syndrome, one can call a defect of genetic material, embedded in stem cells. An example of this genetic damage is Fanconi anemia.
In addition to the above reasons, the etiologic factor of the occurrence of pancytopenia can be assumed enhanced destruction of blood cells. The destruction of blood cells takes place in the spleen - this is a well-known fact. In diseases accompanied by an increase in the spleen (hypersplenism), phagocyte mononuclear cells are activated, which leads to increased destruction of the formed elements of the blood and the development of pancytopenia.
Aplastic pancytopenia is more common in aplastic anemia .
Symptoms of pancytopenia
Pancytopenia manifests itself in three main syndromes: infectious, hemorrhagic and anemia syndrome. The development of all three syndromes is easy to assume, proceeding from the functions of cells, whose fractions are sharply reduced.
The blood platelets (platelets) play an important role in the hemostasis system, their main function is to stop bleeding in the shortest possible time. In addition, platelets provide nutrition to blood vessel cells (endotheliocytes). A sharp decrease in the number of platelets causes deviations in the system of platelet hemostasis, as well as an increase in the fragility of the vessels. All this complex of impaired functions is manifested by an increase in clotting time, i.е. the body needs more time to repair the breach in the vessels, and also bleeding (nasal, uterine, gingival, etc.) that are frequent for no apparent reason. On the skin of such patients often appear bruises arising from the impact of tight elastic bands on clothing, pressing the finger on the skin, due to pinching or as a result of another minor effect on the skin, which in a healthy person does not cause hematoma formation.
Erythrocytes serve as a vehicle for oxygen and carbon dioxide. Thanks to the work of erythrocytes, a constant gas exchange occurs in the tissues. This is a necessary condition for the normal vital activity of the cells of the whole organism. With an insufficient number of erythrocytes in the body, oxygen starvation occurs, which is manifested by dizziness, lethargy, increased fatigue, a person feels broken and tired. Hair begins to fall out, nails become brittle, deformed, muscle weakness is noted, any moderate physical activity leads to an increase in the number of contractions of the heart, increased respiration (dyspnea). Shortness of breath and an increase in the number of heartbeats is an adaptive mechanism by which the body tries to compensate for the lack of oxygen.
Leukocytes and lymphocytes are part of the immune defense. Their insufficiency makes the human body extremely susceptible to microbial and viral diseases. The infected infection often acquires a protracted character and tends to generalize the process. Frequent "visitors" of an organism incapable of fully combating infections are fungi, especially if the patient takes antibacterial drugs or hormonal drugs. In this case, fungal diseases reach their peak.
Deeply dormant herpes virus is also activated.
In autoimmune diseases accompanied by the development of pancytopenia, for example, in systemic lupus erythematosus , besides the indicated hematologic syndrome, there are lesions of the kidneys, skin, heart, etc. caused by the action of autoantibodies.
If pancytopenia accompanies paroxysmal nocturnal hemoglobinuria, then the clinic observes the appearance of black urine.
Diagnosis of pancytopenia
The syndrome of pancytopenia is not difficult to diagnose. It is much more difficult to identify the cause of this condition. The syndrome of pancytopenia is determined using a routine clinical blood test taken from a patient's finger. When calculating the blood elements, their significant decrease is detected, regardless of the type of cells to be detected.
If such a set of abnormalities in peripheral blood is found, the patient should be referred to a specialist for a hematologist who will continue his treatment.
To obtain detailed information about the condition of the red bone marrow, his puncture is performed. Red bone marrow is available for puncturing in the following places: the upper third of the sternum, the crest of the ilium. The procedure for aspiration of the bone marrow is very painful, so local anesthesia is performed before it begins. In order to understand what condition the bone marrow is in, it is enough to get it in a volume of 0.5 ml.
Further, the obtained material is delivered to the laboratory, where the bone marrow composition is evaluated, its ability to repair processes, and also the determination of the genetic sexes of chromosomes in healthy stem and tumor cells of the bone marrow (cytogenetic study). The most unpleasant result of such a study is the detection of bone marrow aplasia, i.e. when the red bone marrow is unable to reproduce normal blood cells. Such a picture of the bone marrow is found in aplastic anemia, when the hematopoietic tissue of the bone marrow is replaced by fat.
Autoimmune pancytopenia gives a positive reaction to Coombs. This test is aimed at identifying autoantibodies on the surface of blood cells.
When suspected of the presence of systemic lupus erythematosus, special lupus cells are found in the patient's blood.
In addition to the diagnostic tests described, urinalysis is performed if there is a suspicion of paroxysmal nocturnal hemoglobinuria. With this disease in the urine, the presence of hemosiderin is detected.
Treatment of pancytopenia
Treatment of pancytopenia should occur in the hematological hospital, in ward-boxes. Patients are recommended to wear disposable masks on a regular basis, for the prevention of stratification of infectious diseases.
Autoimmune pancytopenia is treated by suppressing the patient's immune system, which produces antibodies to its own hematopoietic bone marrow cells. To this end, anti-thymocyte globulin and antilymphocytic globulin are prescribed. Therapy with globulin supplemented by the appointment of Cyclosporine. Globulins and Ciclosporin are the main therapeutic drugs in the autoimmune nature of the disease. In combination with these funds are prescribed glucocorticosteroids, such as Methylprednisolone. The therapeutic effect develops very slowly, often requiring repeated such therapy. This therapy is called immunosuppressive.
If the patient has bone marrow aplasia, in such cases, they are offered to perform bone marrow transplantation. It is believed that the younger the patient, the more likely he is to successfully carry out the procedure and recover. For transplantation it is very important to find a suitable donor of the bone marrow, the most suitable in this regard are siblings. When using donor bone marrow of parents or other relatives, the outcome of treatment is much worse.
To stimulate the bone marrow to reproduce the necessary elements of blood, the patient is injected with colony-stimulating growth factors, such as erythropoietin and granulocyte growth factor (Granocyte, Leucostim, Neupogen).
If the syndrome of pancytopenia is of a tumor nature, then block chemotherapy is used. The drugs are given in a dose, which is calculated based on the patient's age and weight. Calculation of all drugs used for chemotherapy is carried out on cm 2 of the patient's body.
Any therapy of pancytopenia without exception is accompanied by treatment of concomitant diseases, and if necessary substitution therapy in which thrombomass or erythrocyte mass is administered.
? Pancytopenia - which doctor will help ? If a suspected development of this condition should immediately seek the advice of such a doctor as a hematologist.