Hemolytic anemia of Minkowski-Schoffar
To detect the disease, as a rule, is not difficult. The doctor will listen to the patient’s complaints and carry out his examination. Be sure to clarify whether the nearest blood relatives of a person suffered from anemia. In addition to external examination of the skin and mucous membranes, the doctor palpates the abdomen to see if the patient’s liver and spleen are enlarged in size. Mandatory ultrasound for these organs.
Without laboratory tests, the diagnosis can not be confirmed, so the doctor directs the patient to pass the following tests:
Urine analysis. It can detect bilirubin, protein and urobilin in high concentrations.
Blood for biochemical analysis. It will be possible to detect a decrease in cholesterol, 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 decrease in size. In addition, increased ESR, there may be a decrease in platelet and white blood cell count. The color index of the blood will be below normal. To confirm the diagnosis, it is required to study the proteins of the erythrocyte membranes by the method of two-dimensional electrophoresis.
It is necessary to conduct differential diagnosis of Minkowski-Schoffar anemia with autoimmune diseases. First of all, it is necessary to carefully study the family history, since the anemia of Minkowski-Schoffar is most often inherited. In addition, in sick children there are certain changes in the structure of the skull. If the doctor remains in doubt, then the patient is tested by Coombs. When this sample gives a negative result, the diagnosis is confirmed.
Treatment of Minkowski-Schoffar anemia
Depending on the severity of the course of the disease, the methods of its drug correction will differ. During the remission of anemia, therapy is not performed. During an exacerbation of a pathology of the patient it is necessary to be hospitalized.
The treatment is reduced to the following measures:
When the hemoglobin level is reduced to 70 g / l, the patient is transfused with erythrocyte mass.
With a high level of bilirubin in the blood, the patient is administered albumin.
To remove intoxication from the body, the patient is given an infusion therapy.
Cholagogic drugs improve the patient’s well-being in a hemolytic crisis.
The severe course of Minkowski-Schoffar anemia requires surgical intervention. For this, the patient is removed from the spleen. This will not allow you to completely get rid of anemia, but the person’s condition improves, as 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 at the age of under 5 years, since to children splenectomy is fatal. In addition, the absence of spleen in the body contributes to a decrease in immunity. A person becomes susceptible to various infections.
Another operational method for treating anemia is endovascular occlusion. This procedure is an alternative to splenectomy. During the procedure, the patient is given a drug that causes a spleen infarction. Its certain part 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 qualitative and timely treatment, then the prognosis for life is favorable. Removal of the spleen allows you to avoid crises. Without an operation, the average duration of remission between haemolytic crises is 2 years.
Complications of Minkowski-Schoffar anemia
In childhood, Minkowski-Schoffar anemia can cause mental retardation and physical development. Most often this is observed in the case when the child does not receive treatment, or it begins too late.
. In adulthood, the most common complication of Minkowski-Schoffar anemia is cholelithiasis, which develops against the background of an elevated level of bilirubin in the blood. Often, the hemolytic crisis is confused with mechanical jaundice, so adequate therapy is not given to the patient. If the patient has gallstones in the gallbladder, then he is removed along with the spleen.
An Minkowski-Schoffar anemia is a disease that is inherited, so it is impossible to prevent its development. People suffering from this pathology must be registered with a hematologist.
The probability of the birth of a child with hemolytic anemia in a sick mother is equated to 50%. Therefore, such children should be thoroughly inspected from birth.