title: Role of Endothelin-1 in a Syndrome of Myelofibrosis and Osteosclerosis.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.affiliation: 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.
authors.forename: Ralph
authors.initials: R
authors.lastname: Yachoui
authors.affiliation: 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.
authors.forename: Jasmin
authors.initials: J
authors.lastname: Kristianto
authors.affiliation: 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.
authors.forename: Kajal
authors.initials: K
authors.lastname: Sitwala
authors.affiliation: 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.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Blank
mesh_terms: D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D001842:Bone and Bones / Q000378:metabolism* / Q000473:pathology; D019332:Endothelin-1 / Q000378:metabolism*; D005260:Female; D006801:Humans; D008875:Middle Aged; D010026:Osteosclerosis / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D015398:Signal Transduction; D013577:Syndrome
chemical_list: D019332:Endothelin-1