Get ready for the CAMLPR Hematology Test. Use our quizzes, flashcards, and detailed explanations to enhance your understanding. Prepare confidently for your exam!

Multiple Choice

Which syndrome features giant lysosomal granules in white cells and platelets?

Giant lysosome-like granules in white cells and platelets are characteristic of Chediak-Higashi syndrome. This appears because a defect in lysosomal trafficking causes fusion and enlargement of lysosomes inside neutrophils and other leukocytes, as well as in platelets, leading to the distinctive giant granules. Clinically, this finding goes with immunodeficiency and, often, oculocutaneous albinism due to disrupted melanosome transport. The other disorders have different lysosome-related problems without producing the same giant granule change in leukocytes and platelets: Hermansky-Pudlak involves platelet dense bodies deficiency and albinism but not giant lysosomal granules; Griscelli syndrome features pigment abnormalities and immune issues without this leukocyte/platelet granule finding; Wiskott-Aldrich presents with small platelets and eczema with immunodeficiency, not giant lysosomal granules.

Giant lysosome-like granules in white cells and platelets are characteristic of Chediak-Higashi syndrome. This appears because a defect in lysosomal trafficking causes fusion and enlargement of lysosomes inside neutrophils and other leukocytes, as well as in platelets, leading to the distinctive giant granules. Clinically, this finding goes with immunodeficiency and, often, oculocutaneous albinism due to disrupted melanosome transport. The other disorders have different lysosome-related problems without producing the same giant granule change in leukocytes and platelets: Hermansky-Pudlak involves platelet dense bodies deficiency and albinism but not giant lysosomal granules; Griscelli syndrome features pigment abnormalities and immune issues without this leukocyte/platelet granule finding; Wiskott-Aldrich presents with small platelets and eczema with immunodeficiency, not giant lysosomal granules.