Transient erythroblastopenia of childhood


Transient erythroblastopenia of childhood

Description, Causes and Risk Factors:

Abbreviation: TEC.

TEC is a self-limiting disorder of unknown etiology. It is characterized by anemia lasting for few weeks. Although, the review of the literature suggest that it is less common in Europe, with few reported cases in United Kingdom. The onset of TEC occurs at a mean age of 18 to 26 months, although it may occur in infants younger than 6 months or in the children up to the age of 10. The incidence of this disorder is undetermined because it often goes undiagnosed.

The cause of TEC is not known. Research has shown that children with TEC may have an antibody in their blood that reacts against their own baby red blood cells in the bone marrow. Therefore, TEC is a form of “allergy” against one's own red blood cell production. However, TEC is unrelated to other forms of allergies. Children with TEC often have had a virus infection of some sort several months earlier. It is unclear, however, whether this virus is important in triggering the blood problem.

Immunologic and viral causes such as parvovirus B19, human herpes virus have been proposed but not proven. A few familial cases have been reported suggesting genetic predisposition in some individuals.

Researchers looked at the segregation of the markers on chromosome 19q13.2 as this region has been associated with DBA (Diamond-Blackfan anemia). In this region, a mutation of the gene encoding ribosomal protein S19 has been implicated in 25% of DBA cases. The segregation pattern suggested a gene in the 19q13 region may be responsible for TEC, but not the same RPS19 mutation identified in DBA. The segregation patterns also suggested an autosomal dominant pattern of inheritance. The genes involved with the development of TEC therefore still remains unknown.

Symptoms:

Infants and children with TEC are usually quite healthy. Their anemia is often identified on a bloodcount. Some children, however, are noted tobe pale for avariable period of time. Sometimes the paleness is noticed gradually over several months, whereas onother occasions it seems to be sudden. Many children with TEC have no symptoms at all - they feelfine. However, as a result of the anemia, some children with TEC are tired and fatigued; they oftensleep more and exhibit less energy than usual. Children with TEC do not have persistent high fevers,jaundice, dark colored urine, or other problems.

Diagnosis:

Investigations include a complete blood count with reticulocyte count and iron studies. Viral serologies may be useful if hemolysis is considered a possible diagnosis. If the diagnosis remains unclear, a bone marrow aspirate may be indicated.

Treatment:

There is no specific treatment for TEC. Vitamins, hormones, and special diets are of no value. Fortunately, TEC gets better by itself! Within a few weeks, the suppression of red blood cell production in the bone marrow ceases, the reticulocyte (retic) count rises, and the hemoglobin increases steadily to normal (11 to 13 gm/dL), along with return of the child's pink color and resolution of the symptoms of anemia. Some children with TEC, whose hemoglobin values drop below 4 or 5 gm/dL, require a single transfusion of red blood cells to give them a “boost” until they resume red blood cell production on their own. This is not a big problem, since with the careful screening of blood donors that has become standard. The risk of complications from transfusions is negligible.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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