Transient Erythroblastopenia of Childhood

Updated: May 10, 2017
  • Author: Lennox H Huang, MD, FAAP; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Overview

Background

Transient erythroblastopenia of childhood (TEC) is a slowly developing anemia that occurs in early childhood and is characterized by a gradual onset of pallor. As the name suggests, all patients with transient erythroblastopenia of childhood recover completely without sequelae.

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Pathophysiology

The etiology of transient erythroblastopenia of childhood is unknown. However, researchers have proposed numerous viral and immunologic mechanisms. At least 2 separate case reports have noted pure red cell aplasia with concomitant human parvovirus B19 infection. [1, 2] However, a prospective case series of 10 patients failed to identify a single viral causative agent for transient erythroblastopenia of childhood. [3]

In vitro studies using serum and immunoglobulin G (IgG) from some patients with transient erythroblastopenia of childhood demonstrated erythroid colony suppression, suggesting an immunologic etiology. Transient erythroblastopenia of childhood is not caused by a lack of erythropoietin. Bone marrow from patients with transient erythroblastopenia of childhood exhibits an absence of red cell precursors.

A recent case report of half siblings with transient erythroblastopenia of childhood and the accompanying literature review suggests that predisposition to transient erythroblastopenia of childhood may be autosomal dominant in nature. [4]

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Epidemiology

Frequency

United States

Attempts to determine frequency of transient erythroblastopenia of childhood are limited by an unknown number of asymptomatic undiagnosed cases.

Mortality/Morbidity

Morbidity relates to the severity of the anemia and diagnostic workup. Children with transient erythroblastopenia of childhood have reportedly presented with high-output shock secondary to profound anemia. [5] Patients with atypical transient erythroblastopenia of childhood may require invasive tests such as bone marrow aspiration or biopsy. Association of transient neurologic deficits may lead the physician to pursue CNS imaging studies or a neurologic consultation.

A study by Schmidt et al of children under age 10 years indicated that a link may exist between breath-holding spells and TEC. The investigators found that nine out of 366 patients with anemia (2.5%) had breath-holding spells, including five out of the 41 anemia patients who specifically had TEC (12.2%). In comparison, there were 321 cases of breath-holding spells found in the total cohort of 443,470 children studied (0.07%). It was also reported that the breath-holding spells resolved in several of the patients treated for anemia. [6]

Sex

The male-to-female ratio is 1.4:1.

Age

The median age of presentation is 18-26 months; however, the disorder may occur in infants younger than 6 months and in children as old as 10 years. In contrast, Diamond-Blackfan anemia tends to present in child younger than 1 year, whereas human parvovirus B19–associated erythroblastopenia typically presents at an older age.

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