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Autoimmune Hemolytic Anemia in Children

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Hemolytic anemia is an unusual disorder that is characterized by the presence of antibodies that attach to the body’s erythrocytes, causing the premature destruction of red cells (hemolysis). There are two types of hemolysis:

  • Intrinsic: the destruction of red blood cells is due to a defect in the red blood cells. Intrinsic hemolysis is often inherited as in the case of sickle cell disease, erythrocytosis, or thalassemia. The conditions produce RBCs that do not live as long as healthy red blood cells.
  • Extrinsic
    Hemolysis: here the red blood cells produced are healthy. However, they are later destroyed after getting trapped in the spleen due to infections and drugs that affect the cells. In severe cases, the destruction occurs during circulation. This form of hemolysis is also known as autoimmune hemolytic and is an anemia associated with causes like lymphoma and medication like sulfa, penicillin, and anti-malarial drugs.

When the rate of hemolysis exceeds the body’s ability to replace the destroyed red blood cells, anemic signs and symptoms begin to manifest. The body responds by mass producing the damaged red blood cells in the bone marrow.

Blood tests will show lots of premature, young red blood cells in the blood called reticulocytes. Coombs test is carried out in kids to determine if the body is producing antibodies to fight against its red blood cells. If it tests positive, then the child is suffering from AIHA.

How the Antibody Forms in the Body

The cause of intrinsic form of anemia
remains unknown. However, it is associated with underlying diseases like thyroid disease, lupus, rheumatoid arthritis and certain immunodeficiency syndrome.

Antibody production against RBCs is also related to respiratory infections such as Mycoplasma Pneumonia. Thus, treating such diseases causes the anemic antibody to vanish.

Occurrence

AIHA is pretty rare in children and often manifests after a recent infection of a viral illness. It occurs throughout one’s childhood. The condition requires prompt action when diagnosed in teenagers as they are likely to have an underlying systemic disease. The number of infected kids is estimated to be less than 0.2/100,000 with the highest figures found in pre-school children.

Symptoms

Symptoms of this condition may vary among children but the most common include:

  • Abnormal paleness.
  • Yellowing of the eyes and the skin.
  • Fatigue and dizziness.
  • Fever.
  • Increased heart rate.
  • Enlarged liver and spleen.
  • Dark-colored urine.

Note that some of these symptoms resemble certain blood conditions and medical problems, hence the need to consult your pediatrician.

Treating AIHA

AIHA can resolve over a few months, but other types become chronic with periods of reoccurrence and remissions depending on what triggered the production of the antibody. Kids aged ten and above may develop chronic AIHA and may require a blood transfusion to remedy the problem.

Note that a transfusion does not correct the underlying cause of the condition but prevents the occurrence of more complications caused by severe anemic symptoms. The most efficient form of treatment for your child depends on several factors:

  • The kid’s health, general health, and medical history.
  • The extent of the disease.
  • The child’ tolerance to particular medication and therapy.
  • Scope and cause of the condition.

Other forms of
treatment involve:

  • Oral
    medication. The drugs act by suppressing the body’s ability to produce the antibody.
  • Intravenous
    drugs to coax the spleen into ignoring the red blood cells coated with antibodies as they circulate.
  • Phoresis
    treatment, which involves washing the blood using a machine that is laced with an antibody magnet to attract and destroy them. This therapy allows the healthy blood cells to get back to the body.
  • Surgical
    removal of the spleen.

Diagnosing AIHA in Kids

The condition may be suspected after a physical examination of your child if he is complaining of a fast heartbeat, has a pale skin or lips, and fatigues easily. The doctor may also order various diagnostic tests:

  • Blood
    tests which measure the reticulocyte and hemoglobin count.
  • Urine
    tests.
  • Bone
    marrow
    biopsy/aspiration where a small amount of bone marrow fluid or tissue from the hip bones is tested for the size, number, and maturity of the red blood cells.

An Example in Real Life

Karma, a seventeen-year-old resident of Paro in Bhutan, was diagnosed with AIHA after her hemoglobin levels reduced to 1.5. She was turning pale and most of her organs were failing.

Neither Karma nor her parents were aware of the ailment, though she always had low hemoglobin count for which she was treated using steroids. Her parents flew her to Kolkata hospital where an intensive therapy was administered for three weeks.

Further diagnosis at the hospital showed that Karma suffered from SLE (systemic lupus erythematosus).

The doctors figured a blood transfusion wouldn’t treat the condition as the antibodies would quickly destroy any RBCs. As such, they put her on chemotherapy to weaken the antibodies and administered plasmapheresis to minimize the antibody load. The therapy worked, and she is now recovering.

 

Conclusion

The diagnosis of AIHA means your child’s activities are disrupted for a greater part of his childhood. He may feel, tired, angry, cranky, and afraid of the frequent tests done on his body. Honest communication about doctor visits, medication and blood tests creates trust and makes them feel secure. You should also encourage him to listen to his body’s cues and report anemic symptoms.