Minkowski-Shoffard Hemolytic Anemia - Causes, Symptoms And Treatment

2024 Author: Josephine Shorter | [email protected]. Last modified: 2024-01-07 17:49

Minkowski-Shoffard hemolytic anemia

Anemia is characterized by a decrease in the level of hemoglobin in the blood. This disease is widespread. People of all ages suffer from it. Often, anemia is diagnosed in childhood.

Anemia is not just one disease. There are many types of pathology. The most common cause of anemia is nutritional errors when the human body is deficient in vitamins and nutrients. It is possible to eliminate this condition with the help of medical correction, however, you first need to clarify the reason for its development.

Minkowski-Shoffard hemolytic anemia is a serious pathology. It poses a danger to human health and life. It is about this type of anemia that will be discussed further.

Content:

• Anemia - what is it?
• What is the distinguishing feature of hemolytic anemia?
• Causes of Minkowski-Shoffard anemia
• Symptoms of Minkowski-Shoffard anemia
• Diagnostics of the Minkowski-Shoffard anemia
• Treatment of Minkowski-Shoffard anemia
• Complications of Minkowski-Shoffard anemia
• Preventive measures

Anemia - what is it?

Anemia is characterized by a decrease in the level of red blood cells and hemoglobin in the blood. In some cases, not only the number of erythrocytes falls below normal, but also their shape changes. As the disease progresses, red blood cells are unable to perform their function.

Anemia develops against the background of other disorders in the body, in itself it does not manifest. Therefore, to get rid of the disease, it is necessary to establish the cause that led to the change in the composition of the blood.

What is the distinguishing feature of hemolytic anemia?

Hemolytic anemia combines several diseases at once, but the reasons for their development are the same. Red blood cells in the blood are destroyed too quickly, so the red bone marrow begins to produce them intensively. As a result, their growth cycle is disrupted. As anemia progresses, red blood cells undergo massive death.

Hemolytic anemias can be passed from parents to children, and can develop during life. Minkowski-Shoffard anemia is a hereditary pathology.

This disease is also called microspherocytic anemia and hereditary spherocytosis. However, the most commonly used term is "Minkowski-Shoffard anemia" after the scientists who first discovered this pathology.

This type of anemia cannot be called rare, it affects every 5000th inhabitant of the planet. Most often, people inhabiting Northern Europe suffer from it. For the first time, Minkowski-Shoffard anemia makes itself felt in childhood. If this disorder is left untreated, the organism as a whole will suffer.

Causes of Minkowski-Shoffard anemia

With Minkowski-Shoffard anemia, the structure of erythrocytes undergoes pathological changes, which affects their functioning. Red blood cells become brittle and break down much more easily. Hemoglobin comes out of them, which circulates in free form in the blood.

If normally erythrocytes have the shape of a biconvex disc, then with Minkowski-Shoffard anemia they become round, which prevents them from penetrating into small blood vessels. When trying to do this, red blood cells are severely damaged and destroyed. Their level in the blood decreases, which causes the development of anemia.

If one of the parents suffered from Minkowski-Shoffard anemia, then this disease will be inherited by the child. Rarely, sick children are born from absolutely healthy parents. If this happens, experts say the cause is damage to the DNA structure. Genes mutate while the baby is in the womb.

Pathogenic factors are:

• Exposure to a woman's body of radioactive or X-ray radiation.
• Poisoning of a pregnant woman's body with salts of heavy metals, drugs, tobacco smoke.
• Postponed viral diseases.

It should be taken into account that these pathogenic factors can provoke not only anemia, but also other serious disorders in the body of a growing fetus. Therefore, you need to be very careful about your health.

Symptoms of Minkowski-Shoffard anemia

The lower the level of normal red blood cells in the blood, the stronger the symptoms of the disease will be. The first signs of pathology develop in a child during the preschool period, or during early school age. Anemia has an undulating course, periods of remission are replaced by periods of exacerbation.

During a hemolytic crisis, there is a massive death of erythrocytes. In this case, all the symptoms are gaining strength. When the crisis is over, the person will feel well. Excessive pallor of the skin and mucous membranes is possible.

The symptoms of a crisis are:

• High body temperature.
• Headaches.
• General weakness and malaise.
• Yellowness of the skin.
• Abdominal cramps and pains.
• Enlargement of the liver in size.
• Inflammation of the spleen.

The disease can also develop in adulthood. The first sign that a person most often pays attention to is yellowing of the skin. Although in some cases the objective symptoms of the disorder may be completely absent. Often, it is possible to diagnose anemia only during an accidental examination, when a person donates blood for analysis.

Diagnostics of the Minkowski-Shoffard anemia

As a rule, it is not difficult to identify the disease. The doctor will listen to the patient's complaints and carry out an examination. It is imperative to clarify whether the person's closest blood relatives suffered from anemia. In addition to an external examination of the skin and mucous membranes, the doctor palpates the abdomen to determine if the patient's liver and spleen are enlarged. An ultrasound scan of these organs is mandatory.

Without laboratory tests, it will not be possible to confirm the diagnosis, so the doctor directs the patient to pass the following tests:

• Analysis of urine. It can detect bilirubin, protein and urobilin in high concentrations.
• Blood for biochemical analysis. In this case, it will be possible to detect a decrease in cholesterol levels, an increase in the level of indirect bilirubin and lactate dehydrogenase.
• Blood for clinical analysis. In this case, an increase in the level of reticulocytes will be detected, the erythrocytes themselves will decrease in size. In addition, ESR increases, and there may be a decrease in the level of platelets and leukocytes. The blood color index will be below normal.

To confirm the diagnosis, you will need to study the proteins of the membranes of erythrocytes by two-dimensional electrophoresis.

It is imperative to carry out differential diagnostics of Minkowski-Shoffard anemia with autoimmune diseases. First of all, it is necessary to carefully study the family history, since Minkowski-Shoffard anemia is most often inherited. In addition, sick children have certain changes in the structure of the skull. If the doctor still has doubts, then the patient is performed the Coombs test. When this test is negative, the diagnosis is confirmed.

Treatment of Minkowski-Shoffard anemia

Depending on the severity of the course of the disease, the methods of its drug correction will differ. During the period of remission of anemia, therapy is not carried out. During an exacerbation of the pathology, the patient must be hospitalized.

Treatment is reduced to the following activities:

• When the hemoglobin level drops to 70 g / l, the patient is transfused with erythrocyte mass.
• With a high level of bilirubin in the blood, the patient is injected with albumin.
• To remove intoxication from the body, the patient is given infusion therapy.
• Choleretic drugs can improve the patient's well-being during a hemolytic crisis.

The severe course of Minkowski-Shoffard anemia requires surgical intervention. For this, the patient's spleen is removed. This will not make it possible to completely get rid of anemia, but the person's condition improves, since the life cycle of red blood cells can be extended.

After removal of the spleen, the patient will no longer face hemolytic crises. However, this procedure is contraindicated before the age of 5 years, since splenectomy in babies can be fatal. In addition, the absence of a spleen in the body contributes to a decrease in immunity. The person becomes susceptible to various infections.

Another surgical method for treating anemia is endovascular occlusion. This procedure is an alternative to splenectomy. During the procedure, the patient is injected with a drug that causes a splenic infarction. A certain part of it does not die, but continues to function, so the patient's condition improves, but the immunity does not fall.

If the disease proceeds easily and the patient receives high-quality and timely treatment, then the prognosis for life is favorable. Removing the spleen avoids crises. Without surgery, the average duration of remission between hemolytic crises is 2 years.

Complications of Minkowski-Shoffard anemia

In childhood, Minkowski-Shoffard anemia can cause mental and physical retardation. This is most often observed when the child does not receive treatment, or is started too late.

In adulthood, the most common complication of Minkowski-Shoffard anemia is gallstone disease, which develops against the background of an increased level of bilirubin in the blood. Often, hemolytic crisis is confused with obstructive jaundice, therefore, the patient is not given adequate therapy. If a patient has stones in the gallbladder, then it is removed along with the spleen.

Preventive measures

Minkowski-Shoffard anemia is a disease that is inherited, therefore it is impossible to prevent its development. People suffering from this pathology need to be registered with a hematologist.

The likelihood of having a child with hemolytic anemia in a sick mother is 50%. Therefore, such children should be thoroughly examined from birth.

The author of the article: Shutov Maxim Evgenievich | Hematologist

Education: In 2013 he graduated from the Kursk State Medical University and received a diploma "General Medicine". After 2 years, completed residency in the specialty "Oncology". In 2016 completed postgraduate studies at the National Medical and Surgical Center named after N. I. Pirogov.