Thrombotic thrombocytopenic purpura.
A 6-year-old boy presented with microangiopathic hemolytic anemia, thrombo-cytopenia, altered sensorium and intractable bleeding. A diagnosis of thrombotic thrombocytopenic purpura was made and the child recovered dramatically after plasmapheresis. Recent developments in the understanding of TTP are reviewed, including the importance of a metaloprotease required to cleave multimers of von Willibrand factor.
Burle
S
S
Departments of Pediatrics, Nephrology, Hematology+, Choithram Hospital and Research Centre, Indore 452 001, India.
Passi
G R
GR
Salgia
P
P
Modi
A
A
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D001323:Autoantibodies / Q000302:isolation & purification; D002648:Child; D006801:Humans; D008297:Male; D008666:Metalloendopeptidases / Q000235:genetics; D045726:Metalloproteases / Q000172:deficiency / Q000235:genetics; D009154:Mutation; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D014841:von Willebrand Factor / Q000276:immunology
D001323:Autoantibodies; D014841:von Willebrand Factor; D045726:Metalloproteases; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human