In this lecture, we’ll briefly
discuss neutropenia in children.
Okay, neutropenia is when you
don’t have enough neutrophils.
And neutrophils are important in
fighting bacteria, in particular,
in the immune system.
Patients with neutropenia, it’s
usually secondary to another problem.
An example would be an infection.
Infections can sometimes cause
neutropenia, especially viruses.
It could be drug-induced neutropenia
from certain medications
such as acyclovir or chronic
use of antibiotics.
Or this could be benign
neutropenia of childhood
which is a brief autoimmune disease where
children attack their own neutrophils,
but then it generally
There are some very rare primary causes
of neutropenia that we
should at least mention.
Cyclic neutropenia is
It results in a cyclic
picture of neutropenia,
say every three weeks and they
often have fevers as well.
Kostmann syndrome is autosomal recessive.
This is an autosomal congenital lack
of ability of making neutrophils.
These children usually have very severe
neutropenia of an ANC of less than 200
and they tend to get very sick.
Shwachman-Diamond syndrome is a syndrome
that often presents with neutropenia.
It is autosomal recessive, but it has
a number of other things as well
such as pancreatic insufficiency,
or failure to thrive and
So there are some rare primary
causes of neutropenia.
Let’s pause for a minute and say
how do we diagnose neutropenia
and in particular we like
to calculate the ANC.
Unlike for medical students, sometimes they
don’t totally understand this calculation
so I’m going to walk
you through it.
Basically, you’re going to
take the white cell count
and you’re going to multiply it times
both your bands and your segs.
So here’s a CBC, the
white count is 5.6.
This patient has 2%
bands and 5% segs.
So it’s 7% total.
and you take 7% of that, this
patient has an ANC of 392.
What is the normal ANC?
Well, it turns out
this is age dependent.
Newborns tend to have a much
higher ANC than older children.
So in the newborn period,
we expect an ANC of 3,000.
In the first two years of life, we
expect a lower ANC of around 1,000.
And then after two years through
adulthood, the normal ANC is 1,500.
So patients who have neutropenia
are usually asymptomatic.
They are at increased risk for other
things though, such as oral ulcers,
chronic mouth sores
Patients with Shwachman-Diamond
syndrome or Kostmann syndrome
are at increased risk for AML
or myelodysplastic syndrome.
How do we manage neutropenia?
Well for those benign
causes, the child with a
virus who happens to be
found with a low ANC,
there is no treatment
Typically, what we’ll do is
we will check again their ANC
a few weeks later to
make sure it’s come up.
And if it’s come up,
there is no problem.
If it’s remaining down all the time,
we might search for
some other problem
and if it’s coming going frequently, we
might think about cyclic neutropenia.
But in the vast majority of cases,
this just gets better on its own.
For patients with chronic issues around
neutropenia, they may benefit from G-CSF.
Granulocyte colony-stimulating factor
naturally increases the
So we tend to give G-CSF especially
in children with bone marrow failure
such as children with cancer.
is Kostmann syndrome
may benefit from an entire
bone marrow transplant.
In fact, that’s curative.
G-CSF is generally good for patients
who have cyclic neutropenia
where they happen
to have a low ANC
and there’s another significant
infection going on.
Most of the time, we don’t treat
them during one of their cycles,
but if there is infection going on which
we feel is worse because of the low ANC,
we may give the G-CSF.
Also, children may benefit
from IVIg or steroids
if there is an autoimmune component to
their neutropenia and if it’s severe.
Keep in mind for febrile infants,
we will give systemic antibiotics
and do a blood culture and make sure
that culture is negative for 24 hours
if they come in with
fever and neutropenia.
So that’s a summary of
neutropenia in children.
Thanks for your time.