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

Chédiak-Higashi syndrome features include which of the following?

Chédiak-Higashi syndrome is characterized by a lysosomal trafficking defect that produces giant granules in neutrophils and oculocutaneous albinism. The combination of albinism of the eyes with dense, oversized neutrophil granules is a classic clue and directly reflects the underlying problem with lysosome-related organelles. This defect impairs normal phagosome-lysosome fusion and immune cell function, explaining the recurrent infections seen in CHS. The other options point to entirely different conditions—ring sideroblasts in sideroblastic anemia, schistocytes in microangiopathic hemolytic processes, and Reed-Sternberg cells in Hodgkin lymphoma—so they don’t fit CHS.

Chédiak-Higashi syndrome is characterized by a lysosomal trafficking defect that produces giant granules in neutrophils and oculocutaneous albinism. The combination of albinism of the eyes with dense, oversized neutrophil granules is a classic clue and directly reflects the underlying problem with lysosome-related organelles. This defect impairs normal phagosome-lysosome fusion and immune cell function, explaining the recurrent infections seen in CHS. The other options point to entirely different conditions—ring sideroblasts in sideroblastic anemia, schistocytes in microangiopathic hemolytic processes, and Reed-Sternberg cells in Hodgkin lymphoma—so they don’t fit CHS.