A Very Pale Three-Year-Old: Case 2 Diagnosis: Transient Erythroblastopenia of Childhood

A very pale three-year-old
A three-year-old previously healthy girl was sent to the emergency department by her family
physician with severe anemia. Ten days prior, she had been unwell with a fever, pallor and
fatigue lasting three days. She had been seen by her family physician once during the episode
and her parents were reassured that she had a viral illness. During the ensuing days, she
remained afebrile but continued to be pale and tired. She was seen again on the day of
presentation by her family physician, who sent her to the emergency department, where a
complete blood count (CBC) revealed a hemoglobin of 45 g/L (normal 110 g/L to140 g/L).
Review of symptoms was negative. Diet history revealed that she was a picky eater who did
not drink excessive amounts of milk and she appeared to have a generally balanced diet.
Physical examination revealed a pale, irritable but consolable girl with mild tachycardia but
otherwise normal vital signs. She had no evidence of heart failure, lym-phadenopathy or
organomegaly, and there was no rash, bruising, or petechiae. A repeat CBC confirmed the
low hemoglobin of 44 g/L with a mean corpuscular volume (MCV) of 86 fL (normal 80 fL to
94 fL) and normal white blood cell and platelet counts. The reticulocyte count was 0.98%
(28.7109). The remainder of her bloodwork, including electrolytes, bilirubin, urate and
lactate dehydrogenase was normal. She was admitted to hospital for observation and further
investigations.
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CASE 2 DIAGNOSIS: TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD
Additional work up for this child included a bone marrow aspiration, which showed a paucity
of red cell precursors with no abnormality in the other cell lines. Because she was previously
healthy, had no signs, symptoms or laboratory parameters consistent with another cause for
anemia, and her hemoglobin recovered within several weeks of presentation, she was given
the diagnosis of transient erythroblastopenia of childhood (TEC).
Anemia is defined as a decrease in hemoglobin of more than two standard deviations below
the mean for age and sex. A term infant for example, has a normal hemoglobin of 150 g/L to
210 g/L followed by a physiological nadir of 100 g/L around two months of age. The
differential diagnosis of childhood anemia can be approached in various ways, beginning
with a thorough history and physical examination. Age, past medical history, and time course
will help to determine whether the anemia is congenital or acquired, acute or chronic. The
CBC gives information on whether one or more cell lines are involved. The MCV
differentiates between microcytic, normocytic, and macrocytic anemia, and finally, the
reticulocyte count helps to identify anemia associated with underproduction of new cells
versus an appropriate increased production in response to the anemia.
The most common scenario in a young child would be a history of excessive milk drinking
and decreased meat intake, a low MCV as well as low iron and ferritin, which would lead one
to think of iron deficiency anemia. A child of African or Caribbean descent, with a family
history of anemia, and recurrent episodes of painful bony crises or life threatening infections,
would need a hemoglobin electrophoresis to aid in the diagnosis of sickle cell anemia.
Hemolytic disorders are characterized by shortened red cell survival and marked increase in
the reticulocyte count. There may be jaundice, splenomegaly, gallstones and a significant
family or neonatal history. Laboratory findings include raised unconjugated bilirubin, urine
urobilinogen and hemoglobinuria, and a decreased haptoglobin. The blood smear would
likely demonstrate abnormal red cell morphology. A useful comprehensive approach to
childhood anemia can be found in Figure 1.
Figure 1)
A comprehensive approach to childhood anemia. G6PD Glucose 6 phosphate dehydrogenase;
MCV Mean corpuscular volume; Rh Rhesus; SLE Systemic lupus erythematosus; TEC
Transient erythroblastopenia of childhood
TEC is an acquired benign red cell aplasia that occurs in previously healthy children under
the age of four years. The anemia is generally normochromic and normocytic and it is
associated with a severe reticulocytopenia. Platelet numbers are usually normal but
neutropenia may occur. The underlying etiology is unknown, however the disease is self-
limiting (resolves within weeks to two months) and does not recur. It frequently follows a
viral illness although no single agent has been identified (HHV-6 and parvovirus B19 have
been suggested in the past).
Cherrick et al (1) prospectively studied 50 consecutive patients with TEC. At presentation,

the age range was five to 44 months, the hemoglobin ranged from 55 g/L to 84 g/L, platelets
were normal or elevated, and 64% were mildly neutropenic. All exhibited normal myeloid
maturation.
Differentiation of TEC from congenital hypoplastic anemias (eg, Diamond-Blackfan

syndrome) may be difficult in the laboratory but is usually possible on the basis of history.
Other disorders associated with red cell aplasia, eg, renal disease and hypothyroidism, may
need to be excluded. Treatment is supportive and packed cell transfusions may be necessary
if the patient is symptomatic while waiting for the bone marrow to start producing red cells
again.
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CLINICAL PEARLS
In addition to a good history and physical examination in childhood anemia, knowing
the MCV and the reticulocyte count helps to identify the most likely etiology of
anemia.
TEC is a rare acquired red cell aplasia of unclear etiology, presenting as a
normochromic normocytic anemia in children, usually younger than four years of age.

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A very pale three-year-old

CASE 2 DIAGNOSIS: TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD

Cherrick et al (1) prospectively studied 50 consecutive patients with TEC. At presentation,

Differentiation of TEC from congenital hypoplastic anemias (eg, Diamond-Blackfan

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