Recognition of sickle cell anemia in skeletal remains of children

Israel Hershkovitz*, Bruce M. Rothschild, Bruce Latimer, Olivier Dutour, Georges Léonetti, Charles M. Greenwald, Christine Rothschild, Lyman M. Jellema

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

55 Scopus citations

Abstract

The present study discusses in detail the osteological changes associated with sickle cell anemia in children and their importance in differential diagnosis. Posterior calcaneal and specific articular surface disruptive metacarpal lesions are diagnostic for sickle cell anemia. Calvarial thickening, tibial and femoral cortical bone thickening, and bowing are of more limited utility in differential diagnosis. Granular osteoporosis, pelvic demineralization and rib broadening are nonspecific. Localized calvarial 'ballooning,' previously not described, may have diagnostic significance. Bone marrow hyperplastic response (porotic hyperostosis) in sickle cell anemia produces minimal radiologic changes contrasted with that observed in thalassemia and blood loss/hemolytic phenomenon. Two other issues, the osteological criteria for discriminating among the anemias and the purported relationship between porotic hyperostosis and iron deficiency anemia, are also discussed. There is sufficient information to properly diagnose the four major groups of anemias, and further, to establish that iron deficiency is only indirectly associated with porotic hyperostosis. The hyperproliferative bone marrow response (manifest as porotic hyperostosis) to blood loss or hemolysis exhausts iron stores, resulting in secondary iron deficiency.

Original languageEnglish
Pages (from-to)213-226
Number of pages14
JournalAmerican Journal of Physical Anthropology
Volume104
Issue number2
DOIs
StatePublished - Oct 1997

Keywords

  • Anemia
  • Bone pathology
  • Children
  • Iron deficiency

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