Aplastic anemia – what is this diseasecauses, symptoms, treatment and prognosis

Aplastic anemia is hereditary or acquired.
blood pathology due to bone stem cell damage
the brain, which develops a deep depression
hematopoiesis. The disease is more common in individuals after 50–55 years old,
practically independent of gender. Children find hereditary
forms of anemia. The diagnosis is set on the basis of clinical
pictures, blood tests and bone marrow punctures.

Aplastic anemia: what is it?

Апластическая анемия

Апластическая (гипопластическая) анемия — это тяжелое
disorder of hemopoiesis (most often of its links), accompanied by
the development of anemic, hemorrhagic syndromes and infectious
complications.

According to the International Classification of Diseases (ICD-10),
aplastic anemia is included in the group of “other anemias” with the code
ICD D61.

Впервые это заболевание было описано Паулем Эрлихом в
1888 at the 21-year-old woman. The term “aplastic anemia” was
proposed by Chauford in 1904. Апластическая анемия — одно
из самых тяжёлых расстройств hematopoiesis. Untreated patients
severe forms of aplastic anemia die for several
months. With modern adequate treatment, the prognosis is sufficient.
good.

Patients with an already developed disease require follow-up
hematologist observation, systematic examination and long-term
supportive therapy.

Causes of disease

This pathology is classified as polyetiologic.
diseases, that is, there are many reasons, one of which
may take a dominant position and provoke a violation in
hematopoietic system in a patient.

Idiopathic aplastic anemia is accompanied by a violation
production of all blood cells: platelets, white blood cells, red blood cells,
lymphocytes.

Aplastic anemia may be congenital in origin.
(associated with chromosomal aberrations) and acquired (developed
during life).

Congenital form is:

  • hereditary – when the disease is transmitted from parents,
    common signs are blood damage, and
    also anomalies of the structure of organs that are congenital
    (Fanconi anemia);
  • Estren-Dameshek disease appears similar to the previous
    signs of disease, but congenital malformations in development
    are observed;
  • red cell partial aplasia or syndrome
    Diamond-Blackfen, the main feature of which is a decrease
    red blood cells.

Exogenous (external) include:

  • Chemical agents – derivatives of benzene, mercury,
    oil products.
  • Physical effects of penetrating radiation.
  • Medicinal products – anti-tuberculosis drugs
    (Isoniazid, PAS), Analgin, cytostatics, sulfonamides, some
    antibiotics (Streptomycin, Tetracycline, Levomycetin).
  • Infection – in some cases it is proved to be connected with the transferred
    infectious diseases (flu, sore throat, mononucleosis),
    hepatitis C viruses have a suppressive effect on blood cells,
    herpes, Epstein-Barr, cytomegalovirus.

Internal causes include:

  • endocrine disorders – a connection with reduced function
    thyroid, cystic ovarian changes in women;
  • immune shifts due to loss in old age
    the regulatory role of the thymus (thymus gland).

Some malicious agents directly affect
bone marrow (ionizing radiation, chemical and medicinal
substances). Others act indirectly through autoimmune
mechanisms (viral hepatitis B).

Symptoms

Slow-progressive disease is characteristic of aplastic anemia.
current with a tendency to transition to the chronic form with periods
exacerbations.

All clinical symptoms observed with aplastic anemia
can be attributed to one of the three main syndromes: anemic,
геморрагического или симптомокоплекса инфекционных complications.
The appearance of signs of a syndrome is a consequence of
oppression of one or several sprouts of blood formation.

Symptoms of aplastic anemia are any signs.
lack of cells in the blood:

  • with a shortage of red blood cells, there is weakness, pallor
    skin on the face, frequent dizziness, intermittent murmurs
    in ears, causeless shortness of breath;
  • if there are not enough platelets, during the slightest skin contact with
    any objects cause bruising, bleeding is also noted
    from the gums and nose;
  • if there are not enough leukocytes in the blood, then the body
    signals this frequent urinary infections, sore throat,
    pneumonia.

Main symptoms:

  • Pale skin, bruising.
  • Dyspnea.
  • Increased body temperature.
  • Weakness, fatigue, dizziness.
  • Tachycardia.
  • Ulcerative stomatitis.
  • Nasal or gingival bleeding.
  • Decreased appetite, sudden weight loss

These are not all clinical signs, as can manifest
aplastic anemia. Symptoms, безусловно, различаются в каждом
specific case. Moreover, they often resemble other
diseases of the hematopoietic system.

Degrees of severity

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Aplastic anemia is divided into 3 degrees severity:

  • очень тяжелая (тромбоцитов менее 20,0х109/л;
    granulocytes less than 0,2×109 / l)
  • тяжелая (тромбоцитов менее 20,0х109/л; гранулоцитов менее
    0,5х109/л), по данным трепанобиопсии – низкая клеточность
    bone marrow (less than 30% of normal)
  • умеренная (тромбоцитов более 20,0х109/л; гранулоцитов
    more than 0.5 x109 / l)

Aplastic anemia that occurs with selective inhibition
Erythropoiesis is called partial red cell aplasia.

Aplastic anemia in children

Апластическая анемия у детей часто развивается вследствие
hereditary pathologies, including this may be
анемия Фанкони.

When the anemic condition of Fanconi in a baby is detected
inherited bone pathological processes (missing first
finger on the hand, the absence or curvature of the radius). To these
defects are increased heart, kidney abnormalities, small
eyes.

To make an appointment with a pediatric hematologist, you must
The following symptoms in a child:

  • weakness, fatigue, lethargy;
  • pallor of the skin;
  • decreased appetite;
  • frequent infectious diseases, including colds;
  • mood swings;
  • frequent causeless bleeding from the nose;
  • non-stop bleeding after a cut or
    injection at blood test;
  • enlarged lymph nodes;
  • in the event of abnormal blood test results (increase
    ESR, increased platelet, white blood cell or red blood cell count,
    reduced hemoglobin and others.).

If a child is suspected, the disease is promptly taken.
diagnostics. Comprehensive examination consists of:

  • blood donation to detect antibodies to blood cells
  • myelogram
  • X-ray, which determines the bone age,
  • cytological examination after passage
    trepanobiopsy.

Diagnostics

The key to a speedy recovery of patients with aplastic
anemia is the early diagnosis of this pathological
condition, adequate assessment of the severity of the patient’s condition, and
individual approach to the selection of appropriate in each
case treatment method.

The clinical picture of the disease can largely
guide the doctor towards anemia, but the diagnosis should
be confirmed or disproved by laboratory tests and
paraclinical studies.

The most valuable additional research is:

  1. Общий анализ крови (ОАК);
  2. Биохимический анализ крови (БАК) — важен для диагностики
    almost all diseases, so it is prescribed in the first
    turn;
  3. Analysis of urine. In urine analysis, blood may appear as
    manifestation of hemorrhagic syndrome (that is, increased
    bleeding) or microorganisms and white blood cells (as a manifestation of
    infectious complications);
  4. Electrocardiography. Diagnose malnutrition of the heart and
    increased frequency of organ contractions;
  5. Sternal puncture – a technique for obtaining bone marrow from
    chest bone to diagnose blood disorders;
  6. trepanobiopsy – method of bone tissue lifetime extraction
    (mainly spongy) for the study of bone marrow.

At the first detection of the disease, the patient is immediately subject to
hospitalization. Only in a hospital can pick up
the correct method of therapy, to determine the necessary
drugs and their dosing.

Лечение апластических анемий

Patients with aplastic anemia are hospitalized in
specialized departments. They are provided with complete isolation and
aseptic conditions to prevent possible infectious
complications. The effective treatment of aplastic anemia
is a complex problem of practical hematology.

Be sure to ensure that the pathology is not
вызвана определённым неблагоприятным воздействием – если
this is so, unfavorable factors before starting treatment
eliminate.

Basic techniques:

  • transfusion of donated blood or individual elements with
    substitute goal;
  • bone marrow transplantation;
  • drugs that activate blood formation.

For the treatment of pathology, accompanied by a low value
red blood cells or platelets, blood transfusions can be used.
The technique does not allow to get rid of aplastic anemia, but
the disease will be taken under the control of specialists, which is positive
will affect the health of the patient.

The most effective method of this pathology,
как апластическая анемия, является пересадка костного
the brain. Это лечение показано даже тем пациентам, у которых
обнаружена тяжёлая форма патологии. It is important that
transplant taken from a donor according to five or more criteria
matched the recipient’s bone marrow. Otherwise
случае возможно отторжение трансплантата.

To restrain the aggression of the immune system are used:

  • glucocorticoids,
  • cyclosporin A,
  • cyclophosphamide and anti-lymphocytic globulin.

Treatment is carried out strictly in the hospital in the department of hematology.
under the control of red blood cells, platelets and white blood cells, as well as
biochemical blood test indicators that reflect
condition of the liver and kidneys.

Forecast

Forecast для жизни, трудоспособности и состояния здоровья
patients with signs of aplastic anemia depends on
degrees severity of the disease and the depth of the decline in the level of vital
important blood cells.

Acquired aplastic anemia is more favorable.
prognosis since in some cases are reversible after
termination of the effect of the damaging factor on the bone marrow.

Ремиссию удаётся получить примерно у половины больных.
Forecast несколько лучше у детей, чем у взрослых.  Much
improved forecasts after the start of the application of radical methods
treatments (bone marrow stem cell transplantation,
use of immunosuppressants). In 50-90% of cases, the use of these
ways you can achieve complete recovery of the patient.

Disease prevention methods

  1. Primary preventive measures for aplastic anemia
    eliminate the impact of negative environmental conditions,
    thoughtless medication, preventing infectious
    pathology and other factors. A patient with an established
    pathology must be observed by a hematologist, systematically
    be examined, to conduct long-term maintenance treatment.
  2. Вторичная профилактика (то есть предупреждение ухудшения
    conditions of patients with an already developed disease) include:
  • long-term follow-up of patients, including
    signs of recovery;
  • long-term (long-term) maintenance treatment.

Aplastic anemia is a dangerous disease requiring a lot
effort from doctors and patient. Pathology is difficult to eliminate, but it is not
means that the patient lost in advance – success depends on his
action and mood.

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