Transient erythroblastopenia of childhood

454 Pathogenesis

Serum and cellular inhibitors of erythropoiesis and defective bone marrow response to stimulating cytokines have been demonstrated. The condition may be idiopathic or associated with viral infection. It is uncommon but not excessively rare and there may be many subclinical cases where a blood count is not done.

Clinical features

• Boys and girls equally affected: age range 6 months to 5 years; most commonly around 2 years.

• Typically a previously well young child presents with symptoms and signs of anaemia, sometimes but not invariably following an infection. Onset is insidious and the child becomes listless and pale—or just pale.

• Associated infections are usually viral (EBV, mumps), preceding the onset of TEC by some weeks.

• Pallor may be striking.

• No lymphadenopathy or hepatosplenomegaly.

• No physical abnormalities.

Laboratory diagnosis

• Normocytic, normochromic anaemia which may be severe (Hb <5g/dL).

• Reticulocytes absent unless in early recovery phase; WBC and platelets usually normal.

• Blood film shows no abnormality other than anaemia.

• Biochemical profile normal.

• Bone marrow shows normocellular picture with absent erythroid precursors. Iron content is normal.

• No karyotypic abnormalities.

• Exclude parvovirus infection (ffl p454).

• No other investigation is of diagnostic help.

Differential diagnosis

• Exclude acute blood loss and anaemia of chronic disease.

• Diamond-Blackfan anaemia (see previous section). Usually presents within the first 6 months of life and other abnormalities (skeletal malformation, short stature, abnormal facies) are commonly present.

• Parvovirus infection (see above).

Treatment

Blood transfusion should be avoided but may be necessary if symptomatic. Natural history

Spontaneously remits (if not then diagnosis probably wrong) commonly within 4-8 weeks but may be up to 6 months. Relapse is rare. There are no long-term sequelae or associations.

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