“…Thus, it is plausible that changes in the well orchestrated events of EO that affect the hypertrophic chondrocyte-to-trabecular bone transition may likewise affect the unique marrow environment, leading to hematopoietic/immune disorders. Support can be found in human “immuno-osseous disorders” that link skeletal, hematopoietic and immune defects, and include: cartilage-hair hypoplasia (CHH), Kostmann's syndrome, Shwachman-Diamond syndrome, Schimke dysplasia, Fanconi anemia, Diamond-Blackfan anemia, Dubowitz, Omenn and Barth syndromes, kyphomelic dysplasia, spondylo-mesomelic-acrodysplasia, and adenosine deaminase deficiency [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32], [33], [34], [35], [36]. For example, CHH patients, with mutations in the RNA component of the ribonucleoprotein complex RNase MRP, present with disproportionate short stature and deficient cellular immunity [28], [37], reminiscent of the collagen X murine metaphyseal dysplasia and hematopoietic defects.…”