Role of Endothelin-1 in a Syndrome of Myelofibrosis and Osteosclerosis

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 stimu...

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Published in:The journal of clinical endocrinology and metabolism 2015-11, Vol.100 (11), p.3971-3974
Main Authors: Yachoui, Ralph, Kristianto, Jasmin, Sitwala, Kajal, Blank, Robert D
Format: Article
Language:English
Subjects:
Bone and Bones - metabolism
Bone and Bones - pathology
Bone Marrow - metabolism
Bone Marrow - pathology
Endothelin-1 - metabolism
Female
Humans
Middle Aged
Osteosclerosis - diagnosis
Osteosclerosis - metabolism
Osteosclerosis - pathology
Osteosclerosis - physiopathology
Primary Myelofibrosis - diagnosis
Primary Myelofibrosis - metabolism
Primary Myelofibrosis - pathology
Primary Myelofibrosis - physiopathology
Signal Transduction
Syndrome
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Summary: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.
ISSN:0021-972X
1945-7197
DOI:10.1210/jc.2015-2729