Searchable abstracts of presentations at key conferences on calcified tissues
Bone Abstracts (2013) 1 W4.3 | DOI: 10.1530/boneabs.1.W4.3

ECTS2013 Workshops Osteoclast activity and haematopoiesis (3 abstracts)

Osteoclasts and hematopoietic stem cell transplantation in clinical practice

Ansgar Schulz


University Medical Center Ulm, Ulm, Germany.


Dysfunctions of osteoclasts are the pathophysiological hallmark of osteopetrosis (OP). OP is a group of rare inherited human diseases caused by mutations in at least seven different genes. All OP forms are clinical characterized by enhanced bone density. The most severe form infantile OP usually presents with hematological impairment, in particular anemia and thrombocytopenia associated with extra medullary hematopoiesis, leukocytosis and hepatosplenomegaly.

Treatment of OP has to consider the variable phenotype and the involvement of multiple organ systems. Symptomatic conservative treatment in less severe cases has to deal with disturbed calcium homeostasis (usually hypocalcemia with secondary hyperparathyreodism) and bone metabolism leading to pathological fractures. In severe infantile cases, hematopoietic stem cell transplantation (HSCT) is a curative approach, since osteoclasts are derived from the hematopoietic progenitor cell compartment. HSCT in OP is associated with specific complications as for instance delayed hematopoietic engraftment and venous occlusive disease. Furthermore, severe hypercalcemia may develop after HCT particularly in older patient. Life threatening hyperclacemia can be treated successfully by Denosumab, an inhibiting antibody to RANK ligand. Following successful HSCT most patients are free of hematological problems and pathological fractures, but develop growth retardation and a short stature.

The complex interaction of osteoclasts and hematopoiesis in vivo will be illustrated by typical cases of patients with OP before and after HSCT. In particular actual treatment options and site effects will be discussed.

Volume 1

European Calcified Tissue Society Congress 2013

Lisbon, Portugal
18 May 2013 - 22 May 2013

European Calcified Tissue Society 

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