Current insight into thrombotic thrombocytopenic purpura.
During the last three decades knowledge regarding the pathophysiology of thrombotic thrombocytopenic purpura (TTP) has dramatically increased. The discovery of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) deficiency in a subset of patients with TTP has been an important milestone. Apart from this, the use of therapeutic plasma exchange has reduced mortality rates in TTP from 80-90% to 10-20%. Nevertheless, TTP remains a possibly lethal disorder, in which early recognition of symptoms remains extremely important. In the last few years some interesting new insights into TTP have arisen. Firstly, promising reports on rituximab in the treatment of refractory and relapsing cases of TTP have been published. Secondly, risk stratification by means of ADAMTS13 deficiency and ADAMTS13 antibodies might lead to a more tailored approach in treating TTP patients.
University Hospitals Gasthuisberg, Department of Internal Medicine, Leuven, Belgium.
Len
L
Verbeke
Michel
M
Delforge
Daan
D
Dierickx
D051722:ADAM Proteins / Q000172:deficiency / Q000235:genetics / Q000276:immunology; D000071120:ADAMTS13 Protein; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology; D003131:Combined Modality Therapy; D042241:Early Diagnosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010951:Plasma Exchange; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology / Q000235:genetics / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D000069283:Rituximab; D057049:Thrombotic Microangiopathies / Q000145:classification / Q000503:physiopathology
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human