Ralph Yachoui1, Jasmin Kristianto1, Kajal Sitwala1, Robert D Blank1. 1. Division of Rheumatology, Department of Medicine (R.Y.), Department of Pathology (K.S.), Marshfield Clinic, Marshfield, Wisconsin 54449; Endocrine and Reproductive Physiology Program (J.K., R.D.B.), University of Wisconsin, Madison, Wisconsin 53706; Division of Endocrinology, Metabolism, and Clinical Nutrition, Department of Medicine (R.D.B.), Medical College of Wisconsin, Milwaukee, Wisconsin 53226; and Division of Endocrinology, Medical Service (R.D.B.), Clement J. Zablocki Veterans Administration Medical Center, Milwaukee, Wisconsin 53295.
Abstract
CONTEXT: 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. CASE DESCRIPTION: 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. CONCLUSIONS: 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.
CONTEXT: 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. CASE DESCRIPTION: 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. CONCLUSIONS: 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.
Authors: Gregory A Clines; Khalid S Mohammad; Yongde Bao; Owen W Stephens; Larry J Suva; John D Shaughnessy; Jay W Fox; John M Chirgwin; Theresa A Guise Journal: Mol Endocrinol Date: 2006-10-26
Authors: Jasmin Kristianto; Michael G Johnson; Rafia Afzal; Robert D Blank Journal: Endocrinol Metab Clin North Am Date: 2016-11-26 Impact factor: 4.741