I
came across a case of severe congenital neutropenia this morning. This is a
nasty condition that causes affected individuals to be prone to recurrent
infections because of a deficiency of neutrophils.
The
neutropenia is apparent at birth or soon afterward. It leads to recurrent
infections beginning in infancy, including infections of the sinuses, lungs,
and liver. Affected individuals can also develop fevers and inflammation of the
gums (gingivitis) and skin. Approximately 40 percent of affected people have
decreased bone density (osteopenia) and may develop osteoporosis
In
people with severe congenital neutropenia, these bone disorders can begin at
any time from infancy through adulthood.
Approximately
20 percent of people with severe congenital neutropenia develop leukemia or myelodysplastic
syndrome during adolescence.
Some
people with severe congenital neutropenia have additional health problems such
as seizures, developmental delay, or heart and genital abnormalities.
It
turns out there are half a dozen different forms of the condition…
https://ghr.nlm.nih.gov/condition/severe-congenital-neutropenia
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