Giant granules in all granulated cells are characteristic of which primary immunodeficiency disorder?

Giant granules in all granulated cells are a hallmark feature of Chediak-Higashi Syndrome. This rare genetic disorder is characterized by the failure of lysosomal trafficking, leading to the formation of large granules in various immune cells, including neutrophils, eosinophils, and basophils. These abnormal granules impair the cells' ability to effectively respond to infections and carry out their normal immune functions.

The presence of giant granules affects the function of these immune cells, resulting in increased susceptibility to infections and other clinical manifestations, including partial oculocutaneous albinism and neurological issues. Recognizing these giant granules through microscopic examination of blood smears can aid in the diagnostic process for Chediak-Higashi Syndrome.

In contrast, the other listed disorders do not present with this specific characteristic. For instance, Severe Combined Immunodeficiency (SCID) is primarily marked by the absence of functional T and B lymphocytes, while Chronic Granulomatous Disease involves defects in the neutrophils’ oxidative burst, and Hyper-IgE Syndrome is characterized by elevated IgE levels and recurrent skin and lung infections but not by the presence of giant granules. Thus, Chediak-Higashi Syndrome