[Activity loss of Von Willebrand factor cleaving protein (ADAMTS-13) is diagnostic for primary and pregnancy-related thrombotic thrombocytopenic purpura].
OBJECTIVE
To determine whether the measurement of the Von Willebrand factor cleaving protease ADAMTS-13, such as is carried out at the University Medical Centre of Utrecht, The Netherlands, contributes towards the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura (TTP).


METHODS
Descriptive.


METHODS
In a group of 98 patients from 21 hospitals, with a Coombs-negative haemolytic anaemia and thrombocytopenia, the ADAMTS-13 activity was measured. Treatment was given irrespective of ADAMTS-13 activity.


RESULTS
ADAMTS-13 activity was absent in 27 of 29 patients diagnosed with primary TTP and in all 5 pregnancy-TTP patients. In patients suffering from TTP after bone marrow transplantation (post-BMT) and in all other patients included in this study, ADAMTS-13 activity was normal. Of the 32 patients with absent ADAMTS-13 activity, 28 underwent plasmapheresis. This treatment proved effective as all 28 patients responded well. 17 patients with normal ADAMTS-13 activity also underwent plasmapheresis; 5 (30%) responded well to treatment. In 2 cases a final diagnosis of primary TTP was made, in a further 2, haemolytic uraemic syndrome and in 1 case sepsis was diagnosed.


CONCLUSIONS
In this study, the absence of ADAMTS-13 activity predicted primary TTP and TTP of pregnancy with a sensitivity of 93% and a specificity of 100%. Absence of ADAMTS-13 activity is a strong indication for plasma exchange.
Hulstein
J J
JJ
Afd. Hematologie, Universitair Medisch Centrum Utrecht, Utrecht.
Rison
C N
CN
Kappers-Klunne
M C
MC
Hene
R J
RJ
Franx
A
A
de Groot
Ph G
PG
Brand
A
A
Fijnheer
R
R
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016026:Bone Marrow Transplantation; D005260:Female; D017359:HELLP Syndrome / Q000175:diagnosis / Q000201:enzymology; D006801:Humans; D008297:Male; D008666:Metalloendopeptidases / Q000378:metabolism*; D008875:Middle Aged; D011237:Predictive Value of Tests; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis / Q000201:enzymology*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000201:enzymology*; D012680:Sensitivity and Specificity; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human