Congenital Hemolytic Anemias in Children

Hemolytic anemias are common congenital blood diseases. They result from genetic causes and runs in certain families and races. Congenital hemolytic anemias can appear since the moment the child was born but some of them just appear days, months or even years later, specially in mild forms. They have the usual symptoms of anemia plus other symptoms and signs characteristic for each type.

The patient suffers from the usual general symptoms of anemia such as pallor, easy fatigue, and difficulty in concentration , etc but with the addition of the symptoms of hemolysis of red blood cells (jaundice, enlarged spleen, etc) and more symptoms specific for each type of hemolytic anemia. For example, Thalassemia patients have a characteristic facial appearance due to changes that occur in the bone in many cases specially those detected late. Other types such as sickle cell anemia has some characteristic complications and signs such as leg ulcers and other infarctions and a crisis known as thrombotic crisis or (sickle cell crisis).

Not all types of congenital hemolytic anemias are discovered soon after birth. Some types are detected later during infancy or even in childhood after exposure to stress or some agents. The best example is G6PD deficiency anemia which is sometimes detected later in life after exposure to certain chemicals, meications or to the fava beans (by ingestion or sometimes by inhalation).

The blood tests will show the general anemic picture of decreased RBCs numbers and decreased hemoglobin content. Further investigations are needed to reach the exact diagnosis and to identify the type of this congenital hemolytic anemia.

Sickle cell anemia shows a special type hemoglobin called HbS, which is detected by hemoglobin electrophoresis, while thalassemia shows an abnormal high percentage of the fetal hemoglobin HbF. Babies are born with more HbF in their blood and a transition occurs six months after birth from fetal hemoglobin (HbF) to the adult type of hemoglobin (HbA), in thalassemia, this doesn't occur as the body is unable to produce HbA. There are also more tests to detect other types such as microspherocytosis.

In G6PD deficiency, the family should help their child avoid the ingestion of fava beans and should be aware of the drugs that can cause an attack such as certain types of antibiotics and antimalarials.

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