Haematology Vignettes – 10

Transient erythroblastopenia of childhood (TEC)

A 3-year-old child presented with pallor and lethargy. A full blood count was performed and the following results were noted:

Hb 43 g/L, MCV 75.3 fL, WCC 5.3 x 109/L and platelet count 302 x 109/L.

Although the white cell count was normal, the differential revealed a mild neutropenia of 1.0 x 109/L.

A haemolytic screen was performed: the reticulocyte count was 0.1%, the bilirubin

10 umol/L and the serum lactate dehydrogenase 202 IU/L. Initial serology testing for parvovirus B19 infection was negative.

A bone marrow examination revealed a mildly hypocellular marrow with marked erythroid hypoplasia. Myeloid maturation, despite a mild neutropenia, was normal.

As the Hb and reticulocyte count improved spontaneously within a few days, a diagnosis of ‘transient erythroblastopenia of childhood’ was made retrospectively on this child.

Transient erythroblastopenia of childhood

Peripheral blood showing an absence of reticulocytes

Peripheral blood showing an absence of reticulocytes

Bone marrow showing erythoid hypoplasia

Bone marrow showing erythoid hypoplasia

There are 3 major causes of red cell aplasia occurring in childhood. They are namely:

  1. Diamond-Blackfan anaemia (DBA) or pure red cell aplasia
  2. Transient erythroblastopenia of childhood (TEC)
  3. Acute aplastic crisis superimposed on chronic haemolytic anaemia. This form of red cell aplasia may occur in adults as well as in children.

In 1970, Wranne described 4 children with temporary red cell aplasia. This phenomenon became known as ‘transient erythroblastopenia of childhood’ or TEC. TEC commonly presents at 2 years of age, although it can present as young as 6 months of age. The clinical presentation of TEC is essentially normal except for the appearance of pallor and signs of anaemia such as tachycardia.

The Hb levels are variable with a mean of 56 g/L and a reticulocyte count below 1.0%. If the patient is already in the recovery phase, which frequently occurs, the reticulocyte count will be higher. TEC is often associated with a neutropenia.

Bone marrow examination reveals a normocellular marrow with erythroid hypoplasia. Myeloid maturation is normal despite the frequent association with neutropenia.

Serum lactate dehydrogenase, bilirubin and serum haptoglobin levels are normal in TEC and there is usually no evidence of an associated parvovirus B19 infection.

Recovery is denoted by a rise in the reticulocyte count, an increase in the MCV and RDW and a reversal of the neutropenia. Spontaneous, complete recovery, usually occurs within one month of presentation.

There is no specific treatment for TEC. The administration of intravenous IgG or corticosteroids is not indicated.

Wranne L. Transient erythroblastopenia in infancy and childhood. Scand J Haematol 1970; 7:76-81