on 2.9.08 with 0 comments

  • osteoclasts fail to resorb bone where it should be resorbed, leading to a lot of bone where it shouldn’t be

  • characteristically, the femur assumes an Erlenmeyer flask appearance; also, transverse striations on the bone are seen

  • the hematopoietic system and bone marrow fail in these patients, and they have anemia and even thrombocytopenia

  • normally, we would think this very dense bone is very strong, but it is actually quite the opposite. biomechanics of these bones are very abnormal, and patients are paradoxically subject to multiple fractures

  • with the advent of BMT, lots of these patients live for a long time

Category: Pathology Notes



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