Marrow transplantation for juvenile osteopetrosis.

Abstract

Two children with the juvenile form of osteopetrosis were treated with marrow transplants from their HLA identical siblings. Following transplantation each child exhibited extensive bone reabsorption with a marked augmentation of osteoclastic function attributable to donor osteoclasts, including remodeling of bone with expansion of intramedullary hematopoiesis and correction of associated abnormalities of thymic factor and natural killer cells. Osteopetrosis ultimately recurred in one patient in whom engraftment of donor hematopoietic elements was not achieved. Our studies indicate that marrow transplantation will correct osteopetrosis but that permanent reconstitution necessitates sustained engraftment of marrow precursors of cells with osteoclastic activity.

• Authors: M Sorell, N Kapoor, D Kirkpatrick, J F Rosen, R S Chaganti, C Lopez, B Dupont, M S Pollack, B N Terrin, M B Harris, D Vine, J S Rose, C Goossen, J Lane, R A Good, R J O'Reilly
• Journal: The American journal of medicine (Am J Med) Vol. 70 Issue 6 Pg. 1280-7 (Jun 1981) ISSN: 0002-9343 [Print] United States
• PMID: 7015858 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Thymic Factor, Circulating
• Calcium

Topics

• Bone Marrow Transplantation
• Calcium (metabolism)
• Child
• Female
• Hematopoiesis
• Histocompatibility Testing
• Humans
• Infant, Newborn
• Killer Cells, Natural (physiopathology)
• Lymphocytes (physiopathology)
• Male
• Osteoclasts (physiopathology)
• Osteopetrosis (physiopathology, surgery)
• Thymic Factor, Circulating (physiology)
• Transplantation, Homologous