Introduction:
The Coronavirus 19 (COVID-19) pandemic has had significant implications
for patients with chronic medical conditions, particularly those who are
immunocompromised due to malignancy or bone marrow failure (BMF).
Shwachman-Diamond syndrome (SDS) is an inherited BMF disorder
characterized by cytopenias, particularly neutropenia, exocrine
pancreatic dysfunction, and predisposition to myeloid malignancies. Its
manifestations are variable and can affect many body systems, including
the skeletal, cardiac, endocrine, nervous, hepatic, and immune systems.
In most cases SDS is due to recessive mutations in the
Shwachman-Bodian-Diamond Syndrome (SBDS ) gene located on
chromosome 7q11. Because patients with SDS can experience neutropenia
due to BMF, they carry a higher risk for serious infections compared to
the general population; however, it has been unknown whether this
predisposes them to developing COVID-19 or experiencing greater
complications related to COVID-19.