Hemolytic anemia – what it is, causes,diagnosis and treatment

Hemolytic anemia is an independent disease.
blood or pathological condition of the body in which
destruction of circulating red blood cells occurs
various mechanisms.

In normal operation, the natural breakdown of red blood cells
observed after 3 – 4 months from the moment of their birth. With
hemolytic anemia, the decay process is greatly accelerated and
makes only 12 – 14 days. This article will talk about the reasons
of this disease and the treatment of this difficult disease.

What is hemolytic anemia

Гемолитическая анемия

Hemolytic anemia is anemia due to impaired
life cycle of red blood cells, namely the predominance of their processes
destruction (erythrocytolysis) over the formation and maturation
(erythropoiesis). Erythrocytes – the most numerous
a variety of human blood cells.

The main function of red blood cells is the transport of oxygen and oxide
carbon. The composition of these cells includes hemoglobin – protein
involved in metabolic processes.

Erythrocytes in humans function in the blood for a maximum of 120 days, in
average 60-90 days. Aging of red blood cells is associated with a decrease in
the formation of ATP in the erythrocyte during glucose metabolism
in this blood cell

The destruction of red blood cells occurs constantly and is called –
hemolysis. The released hemoglobin breaks down into heme and globin.
Globin is a protein that returns to the red bone marrow and serves
material to build new red blood cells, and is separated from the heme
iron (also reused) and indirect bilirubin.

To determine the content of red blood cells, you can use a blood test,
which is performed during routine medical checks.

According to world statistics, in the structure of morbidity among
blood pathologies, the share of hemolytic conditions is not
less than 5%, of which hereditary hemolytic types prevail
anemia.

Classification

Hemolytic anemias are classified as congenital and
acquired.

Congenital (hereditary)

Due to the impact of negative genetic factors on
эритроциты развиваются наследственные гемолитические anemia.

Currently, there are four subtypes of the disease:

  • non-fermentative hemolytic anemia. In this case, the cause
    destruction of red blood cells is the defective activity of enzymes,
    responsible for their life cycle;
  • hemolytic anemia of Minkowski-Chauffard, or
    microspherocytic. The disease develops due to mutations in the genes,
    responsible for the formation of the proteins that make up the walls
    red blood cells.
  • erythrocyte membranopathy – increased degradation associated with
    a genetically determined defect in their shell;
  • thalassemia. This group of hemolytic anemia occurs due to
    violations of the process of hemoglobin production.

Withобретенные

Occurs at any age. The disease develops
gradually, but sometimes begins with an acute hemolytic crisis.
Patient complaints are usually the same as in congenital form and are related
mainly with increasing anemia.

  • Jaundice is mostly mild, sometimes it is noted only
    subikterichnost skin and sclera.
  • The spleen is enlarged, often dense and painful.
  • In some cases, the liver is enlarged.

Unlike hereditary, acquired hemolytic anemias
develop in a healthy body due to exposure to red blood cells
any external reasons:

  • caused by mechanical damage to the red membrane
    blood cells – cardiopulmonary bypass, prostheses
    heart valves;
  • resulting from chemical damage to red blood cells – in
    lead intoxication, benzene, pesticides, and
    after snake bites.
  • excessive exposure to chemicals (including
    medicines) or hypersensitivity to them;
  • some bacterial or parasitic infections (for example,
    malaria (a disease transmitted by mosquito bites), food
    toxic infections, etc.);

Withчины

Гемолитическая анемия и причины развития

Hemolytic anemias are congenital and acquired, and in
half of the cases – idiopathic, that is, having an unclear
origin when doctors cannot determine the exact cause
development of the disease.

Factors provoking the development of hemolytic anemia,
quite a lot:

  • Blood transfusion incompatible by group or by
    rhesus;
  • Exposure to toxic substances;
  • Congenital heart defects;
  • Leukemia is a variety;
  • Activity of parasitic microorganisms;
  • Chemical and medicinal agents.

In some cases, establish the reason for the development of acquired
hemolytic anemia fails. Such hemolytic anemia
called idiopathic.

Symptoms of hemolytic anemia in adults

The symptomatology of the disease is quite extensive and depends largely on
причины, вызвавшей ту или иную разновидность гемолитической anemia.
The disease can occur only during periods of crises, and outside
exacerbations do not show themselves.

Withзнаки гемолитической анемии возникают только тогда, когда
there is a clear imbalance between the proliferation of blood cells
erythrocyte series and the destruction of red blood cells in the flow
circulating blood, while the bone marrow compensatory function
brain depleted.

Classic symptoms of hemolytic anemia develop only
during intracellular hemolysis of erythrocytes and presented
anemic, icteric syndromes and splenomegaly.

For hemolytic anemia (sickle, autoimmune,
nonferferrate and others) are characterized by the following symptoms:

  • hyperthermia syndrome. Most often, this symptom occurs when
    progression of hemolytic anemia in children. Temperature
    indicators increase to 38 degrees;
  • jaundice syndrome. Associated with enhanced erythrocyte decay, in
    resulting in the liver having to recycle excess
    the amount of indirect bilirubin in a bound form coming into
    intestine, which causes an increase in the level of urobilin and
    sterkobilina. Происходит окрашивание в желтый цвет кожи и
    mucous membranes.
  • Синдром anemia. Это клинико-гематологический синдром,
    characterized by a decrease in hemoglobin per unit volume
    blood.
  • Hepatosplenomegaly is a fairly common syndrome,
    accompanying various diseases and characterized
    an increase in the size of the liver and spleen. Find out what is
    splenomegaly

Other symptoms of hemolytic anemia:

  • Pain in the abdomen and bones;
  • The presence of signs of impaired intrauterine development in children
    (disproportionate characteristics of different body segments,
    malformations);
  • Loose stools;
  • Pain in the projection of the kidneys;
  • Chest pain, resembling myocardial infarction.

Withзнаки гемолитических анемий:

Kinds Description and Symptoms
Non-spherocytic hemolytic anemia The clinical picture of non-spherocytic hemolytic anemia
close to the clinic observed in hereditary spherocytic
form of the disease, i.e., patients have a marked
иной мере желтуха, гепатоsplenomegaly, анемия.

The majority of patients were detected deviations in the state
of cardio-vascular system. Often in the urine crystals were found
hemosiderin, which indicated the presence of a mixed type of hemolysis
of erythrocytes flowing both intracellularly and
intravascular.

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Microspherocytic The disease is congenital, is transmitted by an autosomal dominant
to type. The incidence rate among men and women is the same.
Another name is Minkowski-Chauffard disease or hereditary
spherocytosis.

Sequence of onset of symptoms:

  • желтуха, splenomegaly, анемия.
  • Liver may be enlarged, symptoms of gallstone disease,
    increase sterkobilina and urobilin.
Sickle Cell Sickle Cell анемия — это наследственная
hemoglobinopathy associated with such a violation of the structure of the protein
hemoglobin in which it acquires a particular crystalline
строение — так называемый гемоглобин S. У здорового человека
он представлен типом А.
Thalassemia This is not even one, but a whole group of hereditary diseases.
blood that have a recessive inheritance. Meaning baby
will receive it in the event that both parents give him a patient
gene. In this case, it is said that there is a homozygous thalassemia.
The disease is characterized by the fact that the production of hemoglobin is disturbed,
and he plays a major role in the transport of oxygen through the body.

Some people with thalassemia notice minor
symptoms.

Symptoms:

  • Slow growth and delayed puberty
  • Bone problems
  • Enlarged spleen
Autoimmune Autoimmune гемолитические анемии включают формы заболевания,
associated with the formation of antibodies to their own antigens
red blood cells.

According to the clinical picture, there are two forms of the disease: acute
and chronic.

  • With первой форме у больных внезапно возникает резкая слабость,
    fever, shortness of breath, palpitations, jaundice.
  • With второй форме одышка, слабость и сердцебиение могут
    absent or be mild.
Toxic hemolytic anemia It belongs to the group of hemolytic anemia caused by
by the action of chemical or medicinal agents on
red blood cells.
Membranopathy This is a pathological condition in which defects occur.
shell red blood cells.
Traumatic anemia Mechanical destruction of particles occurs upon collision.
red blood cells with insurmountable barriers. Such a phenomenon is possible with
acute glomerulonephritis, bleeding disorders,
the presence of foreign bodies in the form of artificial valves
hearts.

Как протекает гемолитическая анемия у детей

Hemolytic anemia – a group of different diseases in terms of
its nature, but united by a single symptom – hemolysis
red blood cells.  Hemolysis (their damage) occurs in important
organs: liver, spleen and medulla of bones.

The first symptoms of anemia are not specific and often remain without
attention. Child fatigue, irritability,
crying write off on the load, extra emotion or
features of character.

Children diagnosed with hemolytic anemia
predisposition to infectious diseases, often such children
Included in the group often ill.

With анемии у детей наблюдают бледность кожи, которая возникает
also at insufficiency of filling the bloodstream of the vascular bed,
renal diseases, tuberculous intoxication.

The main difference between true anemia and pseudo-anemia is coloring.
mucous membranes: with true anemia mucous membranes
become pale, with pseudo-anemia – remain pink
(evaluate the color of the conjunctiva).

The course and prognosis depend on the form and severity of the disease, on
timeliness and correctness of treatment, on the degree of immunological
failure.

Complications

Hemolytic anemia may be complicated by an anemic coma. Also
sometimes added to the overall clinical picture:

  • Tachycardia.
  • Low BP.
  • Reducing the amount of urine.
  • Cholelithiasis.

In some patients, a sharp deterioration in the condition causes cold.
It is clear that such people are recommended to stay in
warmer

Diagnostics

With появлении слабости, бледности кожи, тяжести в правом
hypochondrium and other nonspecific symptoms need to be addressed
к терапевту и сдать общий анализ blood. Confirmation of the diagnosis
hemolytic anemia and patient treatment
hematologist.

Determination of the form of hemolytic anemia based on analysis
causes, symptoms and objective data is the responsibility of
hematologist.

  • With первичной беседе выясняется семейный анамнез, частота и
    severity of hemolytic crises.
  • During the inspection, the coloration of the skin, sclera and
    visible mucous membranes, produced by palpation of the abdomen to assess
    the size of the liver and spleen.
  • Spleno- and hepatomegaly confirmed during ultrasound
    liver and spleen.

What tests need to pass?

  • General blood analysis
  • Total bilirubin in the blood
  • Hemoglobin
  • Red blood cells

Comprehensive diagnosis of hemolytic anemia will include the following
research of the affected body:

  • anamnesis data collection, examination of clinical complaints
    the patient;
  • blood test to determine the concentration of red blood cells and
    hemoglobin;
  • determination of unconjugated bilirubin;
  • Coombs test, especially when blood transfusions are needed with
    healthy red blood cells;
  • bone marrow puncture;
  • laboratory serum iron level determination
    method;
  • Ultrasound of the peritoneal organs;
  • study of the form of red blood cells.

Hemolytic anemia treatment

Different forms of hemolytic anemia have their own characteristics and
treatment approaches.

A pathology treatment plan usually includes such
activity:

  1. prescription of preparations containing vitamin B12 and folic
    acid;
  2. гемотрансфузия отмытых red blood cells. To this method of treatment
    resort in the case of reducing the concentration of red blood cells to
    critical indicators;
  3. transfusion of plasma and human immunoglobulin;
  4. to eliminate unpleasant symptoms and normalize size
    the liver and spleen are shown to use glucocorticoid hormones.
    The dosage of these medicines is prescribed only
    врач исходя из общего состояния пациента, а также тяжести
    the course of his illness;
  5. with autoimmune hemolytic anemia, the treatment plan is supplemented
    cytostatics; sometimes doctors resort to surgical procedures
    treat ailment. Most often, splenectomy is performed.

The forecast depends on the cause and severity
diseases.

Any hemolytic anemia that has been started
untimely – a difficult problem. It is unacceptable to try to cope
with her own. Her treatment should be comprehensive and
appointed exclusively by a qualified technician on
based on a thorough examination of the patient.

Prevention

Prevention гемолитических анемий подразделяется на первичную и
secondary

  1. Primary prevention involves measures that prevent
    the occurrence of hemolytic anemia;
  2. Secondary – reducing the clinical manifestations of an existing one
    diseases.

The only possible way to prevent the development of anemia –
maintaining a healthy lifestyle, timely treatment and
prevention of other diseases.

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