Aplastic anemia is characterized by the oppressed process of blood corpuscle production (white blood cells, platelets,and red blood cells) in the bone marrow. This pathology is a rare one.
Its incidence in Western countries is 3-5 cases per million of population a year. Asia has the highest incidence which is up to 15 cases per million of population a year.
There are congenital and acquired forms of aplastic anemia. Congenital forms are characterized by defects in stem cell development and as a result the hematopoiesis (blood cell maturation) disorders appear.
Congenital forms of aplastic anemia are:
Acquired aplastic anemia can occur because of a number of reasons:
In the majority of cases, it’s impossible to establish the cause of the pathology. In such case it is calledidiopathic (unknown origin) acquired aplastic anemia.
The clinical course of aplastic anemia is manifested by three syndromes:
Anemic syndrome appears the first and it is associated with the abnormalities of red cells formation in the bone marrow.
Patients with anemia complain about:
While examination, the pallor skin covering can be noted. Blood flow insufficiency can result in an enlarged liver and swelling in the legs. Heart rate is increased while resting.
Hemorrhagic syndrome is manifested by petechial skin rash. Patients complain about large bruises after minor injuries. Nasal, uterine, and gastrointestinal bleedings can occur.
Infectious and inflammatory syndrome is rare often with bacterial infections. Pneumonia, boils, and abscesses may also develop while having this syndrome.
Diagnosis of aplastic anemia is determined by blood test and a biopsy of the iliac bone. Trepan-biopsy material will reveal the yellow marrow prevalence above the red one.
Diagnosis is done based on the following blood test results:
According to clinical course there are:
Criteria for diagnosis of severe aplastic anemia are:
Super severe aplastic anemia is diagnosed when polymorphonucleocytes level in the peripheral blood drops below 0,2X109/L.
Main goal of the treatment is to reach remission period of aplastic anemia. For some of the patients it’s possible to do it with the help of immunosuppressivetherapy.
Other patients can be treated only with the help of stem cells transplantation. Second aim of the treatment is to normalize blood indicators to block the symptoms of the disease.
Main directions in aplastic anemia treatment are:
If left untreated, there is nothing good for the patient to wait. People die within several months as a result of bleeding or because of infectious complications.
Immunosuppressive therapy can give remission period to the patient with aplastic anemia in 50-70% of cases, though 30% of them will experience the recurrence of the illness. 30-50% of such patients might have myelodysplastic syndromes that are peculiar with hematopoietic (blood formation) inefficiency.
Two-year survival rate of those who underwent symptomatic therapy (if immunosuppressive therapy wasn’t performed or appeared to be inefficient) does not go over 20%.
Best and most effective method of treatment is allogeneic stem cell transplantation. It allows a patient to recover in 70-90% of cases.
Prognosis depends on a number of factors such as: age of patient, the content of reticulocytes in peripheral blood, aplastic anemia severity, presence of concomitant diseases. Low percentage of stem cells transplantation failure is associated with the reaction “graft versus host”.
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