Eurofins Biomnis code

ADAM

Synonyms

• ADAMTS13
• Cleaving protease - Willebrand factor
• PTT
• Purpura thrombotique thrombocytopénique
• Syndrome Upshaw-Schulman
• Upshaw-Schulman syndrome
• vWFCP
• Willebrand factor cleaving protease
• Willebrand factor protease

Specialty

Haematology

Clinical significance

Physiologically, the role of ADAMTS13 is to limit the size (and therefore platelet adhesion) of von Willebrand factor (VWF) multimers, essential for platelet aggregation in the microcirculation. In Upshaw-Schulman syndrome, severe functional ADAMTS13 deficiency occurs (plasma level below 5%), a predisposing factor for the accumulation of large VWF multimers in plasma and therefore the spontaneous formation of platelet thrombi in microvessels. This ADAMTS13 deficiency is hereditary, associated with ADAMTS13 gene mutations (60% substitutions and 40% terminal mutations) along the entire length of the gene and transmitted according to a recessive autosomal mode.

Preanalytics

• 2 x 2 mL :
• citated plasma
•   FROZEN
• Freezing within 4 hours

• A tube specifically for this analysis : No

Further information

Enclose the specific clinical information form (R9-INTGB : ADAMTS13 testing)
Plasma with platelets removed by double centrifugation: 2500 g 15 min and settled plasma. The patient must not receive heparin.
The search for IgG antibodies to ADAMTS'13 is systematically triggered if the activity of ADAMTS'13 is less than or equal to 10%. When the activity is between 10 and 20%, the search for antibodies can be initiated depending on the context and history.

Documents to download

• Clinical data sheet

Methodology

Dosage immunologique en chimiluminescence

Turnaround time

1 week