Aplastic anemia - it is inherited or acquired pathology of the blood caused by the defeat of bone marrow stem cells, which develop as a result of a deep depression of hematopoiesis.The first information about aplastic anemia were obtained in 1888, but as a separate disease entities aplastic anemia was registered only in 1904.
aplastic anemia refers to the most severe violations of blood, as mortality in aplastic anemia reaches the threshold of 80%.
should not be confused aplastichesuyu anemia, which is a separate nosological form with hypoplastic syndrome, which is just one of the many manifestations of various diseases associated with impaired hematopoiesis bone marrow.
Fortunately, the incidence of cases of aplastic anemia is not more than 5 episodes per 1,000,000 population, but the danger of this disease lies in the fact that it affects mainly children and young people age.Gender has no influence on the course and outcome of disease.
Aplastic anemia causes
This pathology is classified as poly etiology of diseases, that is, there are many reasons, one of which can take a dominant position and provoke a disturbance in the hematopoietic system from a particular patient.In addition, the distinction is made between the so-called risk factors, ie, predisposing factors, the totality of which can not only contribute to the development of the disease, but also aggravate the course of the pathological process.Fortunately, in most cases it is possible to identify the causative factor, which was the root cause of symptoms of anemia, but there are situations when it is determined etiopathogenetic factor can not in this case a diagnosis "idiopathic aplastic anemia."
aplastic anemia often plays the role of adverse reactions from the use of certain groups of drugs, and its occurrence is independent of the dose or duration of receiving this drug.The medicines that can provoke a breach of all germs of hematopoiesis in bone marrow include: antihistamines, sulfonamides, tetracycline antibiotics, as well as the preparations of gold.Most often it occurs in aplastic anemia makers levometsitin.
Separately should mention the role of ionizing radiation used in X-ray research methods in causing symptoms of aplastic anemia.This pathology is common among people working in X-ray rooms and in patients undergoing radio wave therapy.
addition, provocateurs aplastic anemia can be a disease of viral etiology in acute (viral hepatitis, cytomegalovirus and herpes infection, HIV).
among hematologists is believed that during pregnancy increases the risk of aplastic bone marrow disorders, but the pathogenetic explanation of this process has not been found.It should be noted that the identification of signs of aplastic anemia is an absolute indication for abortion, and the reported cases of the disease go into remission after artificial delivery.
Another cause of aplastic anemia is a toxic effect on the body of drugs used as chemotherapy for the treatment of cancer pathology, as well as a long stay in a vapor exposure pesticides and insecticides.
Among the causes of hematopoietic function of bone marrow should be considered, and autoimmune diseases in which the immune mechanisms aimed at destroying not only the disease-causing agents, but also on property damage bone cells.
Aplastic anemia symptoms
for aplastic anemia characterized by slow, progressive course with a tendency to become chronic with periods of exacerbation.
All clinical symptoms observed in aplastic anemia can be attributed to one of three major syndromes: anemic, hemorrhagic or infectious complications simptomokopleksa.The appearance of signs of a syndrome is a consequence of the oppression of one or more germ hematopoiesis.
main complaints of patients in the development of symptoms of anemia caused by insufficient levels of hemoglobin and erythrocytes in the peripheral blood are expressed weakness and decreased disability, dizziness and tinnitus in the preservation of hearing, feeling short of breath and heart palpitations seizures.
first signs of occurrence of haemorrhagic syndrome in a patient with aplastic anemia is the appearance of bruises and hematoma on the skin of non-traumatic origin.Patients with aplastic anemia often complain about the nosebleeds, and bleeding gums.In marked inhibition of platelet hemopoietic stem may be bleeding in the brain.
Symptoms of leukocyte reduction as one of the pathogenetic links of aplastic anemia, a predisposition to diseases of infectious nature.These patients are classified as "sickly", that is, within a year they have been more than ten episodes of infectious diseases.Severe depression of white blood cells triggers production of purulent-septic complications (necrotizing sore throat, aphthous stomatitis, otitis, felon, post-infectious abscess, generalized sepsis).
Already during the initial examination of the patient can determine the characteristic symptoms of aplastic anemia: marked pallor and blueness of the skin with the presence of bruising, without clear boundaries, and sometimes punctate petechial rash.Increased hepatic boundaries and edema of lower limbs indicate the development of heart failure.Auscultatory marked tendency to hypotension, sinus tachycardia and rough systolic murmur in all points of auscultation.An important diagnostic feature of aplastic anemia is unaltered in the size of the spleen.
degree of manifestation of a symptom is directly dependent on the degree of inhibition of hematopoiesis germs.Aplastic anemia is severe such diagnostic criteria: neutrophil least 0.5 × 109 / L Platelets 20 × 109 less / l corrected reticulocytes increase of less than 1%.If the patient has at least one of these criteria, combined with a marked reduction in bone marrow cellularity, it is an occasion to establish the diagnosis of the patient "severe aplastic anemia."
Aplastic anemia in children
in childhood often debut types of congenital aplastic anemia, which include Fanconi anemia and Black fan Diamond.
Fanconi anemia is a hereditary pathology, inherited in an autosomal recessive manner, that is, cases of the disease are observed only in families where both parents are carriers of the abnormal gene.Fanconi anemia is manifested not only in the deep oppression of all types of blood, but also the appearance of congenital malformations.
debut of the disease between the ages of 4-12 years, but hematologic problems may arise immediately after birth.For congenital anomalies include bone age discrepancy passport, birth defects of the cardiovascular system, microcephaly, abnormal development of bone, genitourinary system, retarded psychomotor development.Hematologic changes manifest themselves later in life (an average of 9 years to achieve), and the clinical picture is dominated by symptoms of hemorrhagic symptom, after which progressively growing signs of leukopenia and erythropenia.
This pathology is rapidly progressive course, so in the absence of adequate treatment, death occurs within 6 months.The cause of death is often a massive gastrointestinal bleeding or cerebral hemorrhage.Complications of Fanconi anemia is a disease transformation to acute leukemia, as well as oncopathology of the gastrointestinal tract.The only effective treatment for congenital Fanconi anemia is a bone marrow transplant.
Diamond Black fan anemia refers to a group of sporadic and hereditary diseases characterized by lesions of the blood erythrocyte exclusively germ hematopoiesis.The debut of the disease occurs in the first year of life.
Children with anemia Diamond Black fan has a typical phenotype: pale skin, large upper lip, wide-set eyes, dry split ends, the backlog of passport bone age.
characteristic changes in laboratory parameters of peripheral blood is progressing normochromic anemia with full preservation of cell proliferation and platelet-granulocyte series.
This pathology is characterized by a chronic indolent course with a tendency to spontaneous remission.The preferred method of treatment is the use of corticosteroid therapy, as well as life-saving transfusion of packed red blood cells.
in childhood acquired forms of aplastic anemia are very rare and appear the same symptoms as adults in the absence of congenital abnormalities.
If you have a child of a violation of hematopoietic function of bone marrow absolutely necessary to carry out the differential diagnosis of diseases such as acute lymphoblastic and myeloid leukemia, megaloblastic anemia syndrome, hypersplenism, bone marrow metastases.
child with suspected congenital aplastic anemia necessarily subject to comprehensive survey, which includes: after trepanobiopsy cytogenetic analysis, a blood test for the presence of antibodies to red blood cells, bone X-ray of the definition of age, myelogram.Also screening is required for all immediate family members of the sick child.
Aplastic anemia treatment
key to a speedy recovery of patients with aplastic anemia is an early diagnosis of the disease state, an adequate assessment of the severity of the patient's condition, as well as individual approach to the selection of appropriate in each case, the method of treatment.
newly diagnosed aplastic anemia is a cause for hospitalization of the patient to the hospital, regardless of the severity of clinical symptoms and laboratory parameters.
In a situation where known etiopathogenetic factor, trigger the development of aplastic anemia, treatment should start with the elimination of the root causes, in order to prevent further progression of the oppression of hematopoiesis.
as therapeutic measures when diagnosed with aplastic anemia hematologists worldwide use the following methods: Replace the transfusion of whole blood or blood components, bone marrow transplantation, and the use of drugs, aimed at stimulation of hematopoiesis.
replacement therapy of aplastic anemia involves the use of a suitable exchange transfusion of donated blood or some of its elements (platelet, packed red blood cells and granulocytes).This method of treatment is preventative as transfusion only the reservoir of the required elements of the blood, but does not restore hematopoiesis in bone marrow.Moreover, in this form of aplastic anemia is an autoimmune, this method is not applicable, as the introduction of foreign cells body produces an immune response that leads to failure of treatment.
Note that multiple infusions of blood accompanied by excessive accumulation of iron in the body is deposited in the vital organs, disrupting their structure.In this case it is advisable to combine the reception transfusion preparations promote excretion of iron from the body.In order to prevent transplant rejection process before the procedure of bone marrow transplantation the patient is inappropriate to apply the transfusion of blood components.
most effective and at the same time the most dangerous method of treatment of aplastic anemia is a bone marrow transplant, since only this method can be a trigger for the normalization of the function of bone marrow hematopoiesis.This method is preferably used at a young age of the patient after a careful selection of a suitable donor, to whom may be the closest relative of the patient.Preparatory procedures for the transplantation of bone marrow stem cells are the patient's effects on the body of high-dose radiation exposure, and the use of chemotherapy drugs.A preparatory step by a donor blood sampling, filtering it from the healthy stem cells and their introduction into the patient's bloodstream, via which they are discharged into the medullary cavity, where it is the proliferation of healthy blood cells.
To avoid complications, patients received drugs to prevent transplant rejection, however, is not always possible to avoid this fatal situation for the patient.This method of treatment is expensive and requires compliance with certain conditions, which are available only in specialized clinics, and therefore not widely used as a treatment of aplastic anemia.
patients with aplastic anemia of autoimmune origin require the use of immunosuppressive drugs, the effect of which is aimed at suppressing the inappropriate functioning of the immune system.The drugs of choice in a given situation are: Tacrolimus (daily dose of 10 mg / kg p.o.), antithymocyte globulin (daily dose 15 mg / kg i.v.) and antilymphocytic globulin (daily dose of 130 mg intravenously).The use of immunosuppressive drugs in most cases is accompanied by severe side effects, the patient's life-threatening (anaphylactic shock, serum sickness, and infectious diseases), to prevent them appropriate to apply corticosteroids (prednisolone at a daily dose of 1 mg / kg).Undoubtedly, the immunosuppressive therapy is one of the most effective treatments for aplastic anemia, but there are cases when after taking the drugs in this group there is a relapse of the disease.
as adjunctive therapy to the main direction in the treatment of aplastic anemia drugs are used to stimulate red blood cells, so-called colony stimulating factors (Molgramostin, filgrastim, Leykomaks).This group of drugs are effective in stimulating cell proliferation of granulocytic series, so the area of application are cases of aplastic anemia, accompanied by severe leukopenia.Therapy colony stimulating factor is at least two weeks, and the preferred method is parenteral administration of drugs in a daily dose of 5 mg / kg.The limiting factor in the use of drugs in this group is their high cost.
scientifically proven stimulatory effect of androgens on all the germs of blood, and therefore, it is now widely used in the treatment of androgen treatment of aplastic anemia.These drugs are appropriate to apply only in the case of disease among men and recommended the introduction of long courses of drugs (testosterone propionate twice daily to 1 ml of 5% solution or prolonged drug Sustanon - 250 1 ml 1 time per month).
as a palliative treatment using radical splenectomy, which in 85% of cases have a positive effect in the treatment of aplastic anemia.Pathogenetic substantiation of this method is that when you remove the spleen stops blood cell sequestration and sharply limited production of antibodies against its own healthy bone marrow cells.