Vui lòng sử dụng định danh này để trích dẫn hoặc liên kết đến tài liệu: http://dspace.hmtu.edu.vn/handle/DHKTYTHD_123/368
Nhan đề : Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies
Tác giả : Crawley, James T. B.
Scully, Marie A.
Năm xuất bản : 2013
Nhà xuất bản : American Society of Hematology
Tóm tắt : VWF is a multimeric plasma glycoprotein that specifically recruits platelets to sites of vessel injury. VWF multimeric size is central to this function, with larger multimers being more hemostatically active. Regulation of VWF multimeric size is mediated by the plasma metalloprotease ADAMTS13 (ADisintegrin And Metalloproteinase with ThromboSpondin type 1 motifs, member 13). This enzyme can only recognize and cleave VWF when it is unraveled by rheological shear forces of the flowing blood. After the exposure of cryptic exosites, VWF recognition by ADAMTS13 involves multiple interactions that enable the protease to cleave VWF. Loss of VWF multimer size regulation caused by severe ADAMTS13 deficiency (either inherited or acquired) is associated with the microvascular thrombotic disorder thrombotic thrombocytopenic purpura (TTP). The sequelae associated with TTP are widely thought to be linked to hyperreactive circulating VWF that cause unwanted platelet aggregation in the high shear environment of the microvasculature. Diagnosis of TTP is primarily made through a combination of symptoms, analysis of plasma ADAMTS13 activity, and detection of inhibitory anti-ADAMTS13 antibodies. Current frontline treatments for TTP include plasma exchange, which serves to remove inhibitory antibodies (in acquired TTP) and provide a source of functional ADAMTS13, and steroids to treat the autoimmune component of acquired TTP. The use of anti-CD20 therapy has also exhibited encouraging results in the treatment of acquired TTP. Newer therapeutic strategies that are currently being explored or are in development include recombinant ADAMTS13, a hyperreactive ADAMTS13 variant, and anti-VWF therapy. This review discusses the basic biochemistry of VWF and ADAMTS13, their dysfunction in TTP, and therapeutic approaches for the amelioration of TTP.
URI: http://220.231.117.85:8000/handle/DHKTYTHD_123/368
Bộ sưu tậpHuyết học = Hematology

Danh sách tệp tin đính kèm:
Tên tệp tin Mô tả tệp tin Dung lượngĐịnh dạng  
Hematology-2013-Crawley-292-9.pdf
  Restricted Access
809.52 kBAdobe PDFẢnh bìa
 Gửi yêu cầu


Khi sử dụng tài liệu trong thư viện số bạn đọc phải tuân thủ đầy đủ luật bản quyền.