In sickle cell disease, which organ and finding is described by infarcts leading to fibrosis and increased infection risk?

Prepare for the Hematologic, Immunologic, and Neoplastic Disorders Test. Study with flashcards and multiple-choice questions, each with explanations and hints. Get ready for your exam!

Multiple Choice

In sickle cell disease, which organ and finding is described by infarcts leading to fibrosis and increased infection risk?

Explanation:
Vaso-occlusive events in sickle cell disease most critically affect the spleen early in life. Repeated splenic infarcts progressively replace functioning splenic tissue with fibrotic scar, leading to autosplenectomy and functional asplenia. The spleen is essential for clearing encapsulated bacteria, so losing its function dramatically increases the risk of severe infections from organisms like Streptococcus pneumoniae. This combination—infarcts that heal into fibrosis plus a higher infection risk due to loss of splenic function—best fits the described scenario. The other options describe plausible sickle-related problems (kidney concentrating defects from vasa recta damage, priapism from microvascular occlusion, liver involvement with enlargement and scarring) but they don’t capture the classic sequence of infarcts progressing to fibrosis in the spleen with the associated infection risk.

Vaso-occlusive events in sickle cell disease most critically affect the spleen early in life. Repeated splenic infarcts progressively replace functioning splenic tissue with fibrotic scar, leading to autosplenectomy and functional asplenia. The spleen is essential for clearing encapsulated bacteria, so losing its function dramatically increases the risk of severe infections from organisms like Streptococcus pneumoniae. This combination—infarcts that heal into fibrosis plus a higher infection risk due to loss of splenic function—best fits the described scenario.

The other options describe plausible sickle-related problems (kidney concentrating defects from vasa recta damage, priapism from microvascular occlusion, liver involvement with enlargement and scarring) but they don’t capture the classic sequence of infarcts progressing to fibrosis in the spleen with the associated infection risk.

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