A patient with jaundice and hepatomegaly is diagnosed with congenital neutropenia. What is the underlying issue?

The underlying issue in congenital neutropenia is decreased production of neutrophils. This condition often arises from a variety of genetic mutations that affect the development of myeloid cells in the bone marrow, leading to an inadequate number of neutrophils.

In congenital neutropenia, bone marrow production is impaired, which results in a lower than normal number of neutrophils circulating in the bloodstream. This deficiency can lead to increased susceptibility to infections and may also be associated with other manifestations such as jaundice and hepatomegaly, indicative of underlying systemic issues.

The correct answer highlights the fundamental problem of decreased neutrophil production rather than increased destruction, which would pertain to different pathologies, such as autoimmune conditions or malignancies that involve an active process of neutrophil clearance from the circulation. Thus, the focus on production establishes a clearer understanding of congenital neutropenia as a condition where the bone marrow fails to produce sufficient white blood cells needed for immune defense.