We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Role of Endothelin-1 in a Syndrome of Myelofibrosis and Osteosclerosis.
- Authors
Yachoui, Ralph; Kristianto, Jasmin; Sitwala, Kajal; Blank, Robert D
- Abstract
<bold>Context: </bold>Primary myelofibrosis is one of the chronic myeloproliferative disorders characterized by bone marrow fibrosis associated with extramedullary hematopoiesis and osteosclerosis. Endothelin-1 (ET1) is a potent vasoconstrictor that is also a key mediator of osteoblastic bone metastases by stimulating osteoblast proliferation and new bone formation.<bold>Case Description: </bold>We report laboratory, radiographic, bone densitometry, and bone histology data of a patient presenting with newly diagnosed, biopsy-proven myelofibrosis and osteosclerosis. We were able to demonstrate abundant ET1 signaling in the bones of our patient.<bold>Conclusions: </bold>We believe that ET1 is responsible for the osteosclerosis that develops with advanced myelofibrosis and suggest that ET1 signaling may play a role in other osteosclerotic settings as well.
- Publication
Journal of Clinical Endocrinology & Metabolism, 2015, Vol 100, Issue 11, p3971
- ISSN
0021-972X
- Publication type
journal article
- DOI
10.1210/jc.2015-2729