HS – Congenital anemia spherocytic – Hereditary spherocytosis

Spherocytosis (Congenital Spherocytic Anemia; Hereditary Spherocytosis)

Description spherocytosis

Spherocytosis is a disease, which causes the anomaly of erythrocyte membranes. Healthy red blood cells have a flattened shape, with the projection of the edges of the disc, spherocytosis and leads to a spherical form erythrocytes, and premature failure of these cells.

Red blood cells in spherocytosis are small round size, and are more fragile, than healthy red blood cells. Because of the rounded shape, if it enters the spleen, they are destroyed.

Spherocytosis is most common in people of Northern European descent.

Spherocytosis can proceed very gently, with minor symptoms, or be very difficult, with symptoms, are rapidly progressing. These symptoms may occur after certain infections. In severe cases of spherocytosis it can be diagnosed in childhood, whereas with mild symptoms of disease is usually diagnosed in adulthood.

In the treatment of the symptoms can be controlled spherocytosis.

The causes of spherocytosis

Сфероцитоз – наследственное заболевание, induced genes, inherited from parents.

Risk Factors spherocytosis

Factors, which may increase the risk include spherocytosis:

• Have a family member with spherocytosis.

Symptoms spherocytosis

Symptoms include spherocytosis:

• Jaundice;
• Paleness;
• Breathlessness;
• Fatigue;
• Weakness;
• У детей – раздражительность и нервозность;
• Gemoliticheskaya anemia;
• Gallstones.

Diagnosis spherocytosis

The doctor will ask about your symptoms and medical history, and perform a physical examination.

Tests may include the following:

• Examination of the spleen;
• Blood tests;
• Analyses of liver function;
• Осмотические и тесты на хрупкость эритроцитов – чтобы диагностировать наследственный сфероцитоз;
• Реакция Кумбса – анализ антиглобулина, to examine red blood cell antibodies.

Treatment spherocytosis

Treatments include spherocytosis:

Folic acid

1 mg per day of folic acid and optionally, blood transfusion, It recommended during periods of severe anemia.

Operation at spherocytosis

Surgical removal of the spleen can cure the anemia. Pathological forms of blood cells remain, but they will not be destroyed in the spleen.

Currently, a few weeks before splenectomy introduced meningococcal, Hib and pneumococcal vaccine. After surgery, it is recommended Lifetime penicillin prophylaxis, to prevent dangerous infections. The operation is not recommended for children under the age of five years. After the procedure, there is a lifetime risk of serious and potentially life-threatening infections.

Prevention spherocytosis

Because spherocytosis is an inherited disease, it is impossible to prevent. Regular inspection of high-risk individuals spherocytosis can prevent the risk of complications in early treatment.