Which condition is characterized by an abnormality in neutrophil chemotaxis?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Hyper-IgE syndrome, also known as Job's syndrome, is characterized by an abnormality in neutrophil chemotaxis. In this condition, there is a defect in the immune response that leads to high levels of immunoglobulin E (IgE) and recurrent skin and pulmonary infections. The underlying problem is typically associated with mutations in the STAT3 gene, which impacts the signaling pathways that are crucial for the proper functioning of several immune cells, including neutrophils.

In hyper-IgE syndrome, although neutrophils are present, their ability to migrate to sites of infection is impaired. This results in a failure to mount an appropriate response to infections, leading to the characteristic symptoms of recurrent bacterial infections and abscess formation. The decreased chemotactic response makes it difficult for neutrophils to reach sites of inflammation or infection efficiently, contributing to the immunocompromised state of the individual.

The other conditions listed have different mechanisms of immune dysfunction that do not center around neutrophil chemotaxis. For example, chronic granulomatous disease is primarily due to defects in oxidative burst mechanisms of phagocytes, affecting their ability to kill ingested pathogens, rather than their movement. Severe combined immunodeficiency relates to defects in T

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