Back to Search
Start Over
Prospective study on the behaviour of the metalloprotease ADAMTS13 and of von Willebrand factor after bone marrow transplantation
- Source :
- British Journal of Haematology. 134:187-195
- Publication Year :
- 2006
- Publisher :
- Wiley, 2006.
-
Abstract
- Summary Thrombotic microangiopathies (TMAs) are rare but serious complications of bone marrow transplantation (BMT). Clinical manifestations are similar to those of thrombotic thrombocytopenic purpura (TTP), but prognosis is generally poorer despite plasma exchange. The enzymatic activity of the plasma metalloprotease ADAMTS13, which cleaves ultralarge thrombogenic multimers of von Willebrand factor (VWF) derived from activated endothelial cells, is very low or undetectable in patients with classic TTP, and protease deficiency is thought to play a mechanistic role in the formation of platelet thrombi in the microcirculation. This is the first prospective study to evaluate the incidence of TMA in 46 consecutively recruited patients undergoing autologous or allogeneic BMT and explore in parallel the behaviour of ADAMTS13, VWF antigen and VWF multimer size. The incidence of post-BMT TMA was 6% (three of 46); all cases occurred after allogeneic BMT. Compared with baseline values plasma ADAMTS13 activity was significantly reduced in patients undergoing BMT, particularly after the conditioning regimen (mean values: 50 ± 22 vs. 77 ± 32%; P
- Subjects :
- Adult
Male
medicine.medical_specialty
Transplantation Conditioning
Thrombotic thrombocytopenic purpura
ADAMTS13 Protein
Graft vs Host Disease
Gastroenterology
Von Willebrand factor
immune system diseases
hemic and lymphatic diseases
Internal medicine
von Willebrand Factor
Humans
Medicine
Platelet
Prospective Studies
Prospective cohort study
Aged
Bone Marrow Transplantation
Hematology
Purpura, Thrombotic Thrombocytopenic
biology
business.industry
Vascular disease
Middle Aged
medicine.disease
ADAMTS13
ADAM Proteins
medicine.anatomical_structure
Hematologic Neoplasms
Immunology
biology.protein
Female
Bone marrow
business
Subjects
Details
- ISSN :
- 13652141 and 00071048
- Volume :
- 134
- Database :
- OpenAIRE
- Journal :
- British Journal of Haematology
- Accession number :
- edsair.doi.dedup.....4456140f7eeee03066c6e7460abbda51