ADAMTS13 Activity
Clinical Background:
ADAMTS13 is a serine protease which controls the eventual size of the vW multimer that is in the circulation. Deficiencies of ADAMTS13 leads to Ultra Large vWM (UL-VWM) circulating in the bloodstre...
ADAMTS13 is a serine protease which controls the eventual size of the vW multimer that is in the circulation. Deficiencies of ADAMTS13 leads to Ultra Large vWM (UL-VWM) circulating in the bloodstream, the size of these molecules mean that they can cross-link non activated platelets and generate spontaneous thromboses. This can lead to Thrombotic Thrombocytopenic Purpura (TTP) which is a disorder with a potential high mortality. Deficiencies in ADAMTS13 may be congenital (Upshaw Schulman syndrome), which gives a recurring form, or acquired due to formation of auto-antibodies which give an acute disease.
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Test Details
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Discipline:
Haemostasis
Haemostasis
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Specimen Container Adult:
Light blue -sodium citrate
Light blue -sodium citrate
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Specimen Container Paediatric:
Light blue (under clear cap) – sodium citrate
Light blue (under clear cap) – sodium citrate
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Minimum Volume Adult:
2.7 ml
2.7 ml
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Minimum Volume Paediatric:
1.8 mL (whole blood)
1.8 mL (whole blood)
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Special Requirement:
Tube must be correctly filled to line, to ensure correct ratio of blood to anticoagulant, ensure adequate mixing.
Tube must be correctly filled to line, to ensure correct ratio of blood to anticoagulant, ensure adequate mixing.
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Sample Stability:
2 citrated blood samples to arrive in the Blood Sciences laboratory at RVI within 48 hrs of sample collection as uncentrifuged whole blood stored at room temperature.
OR
Double spin citrated plasm...
Read More2 citrated blood samples to arrive in the Blood Sciences laboratory at RVI within 48 hrs of sample collection as uncentrifuged whole blood stored at room temperature.
OR
Double spin citrated plasma at 2000G and separate into 4 aliquots.
Freeze immediately at -70°C. and send frozen on dry ice.
Note: We will not be able to test frozen samples which are defrosted on arrival
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Transport Requirements:
Please telephone the laboratory before sending samples
Samples should be transported to lab without delay
Please telephone the laboratory before sending samples
Samples should be transported to lab without delay
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Freq Analysis:
On request
On request
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Add On Test:
tnu-tr.bloodsciencesadditions@nhs.net (external)
tnu-tr.bloodsciencesadditions@nhs.net (external)
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Quality Assurance:
UKNEQAS
UKNEQAS
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Reference Ranges:
ELISA activity assay: 40 – 130 IU/dL
AcuStar activity assay: 60 – 130 IU/dL
ELISA activity assay: 40 – 130 IU/dL
AcuStar activity assay: 60 – 130 IU/dL
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Routine Contact Name:
RVI or Freeman Section Leaders
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Routine Telephone:
RVI: 0191 2820332, Freeman: 0191 2231195
RVI: 0191 2820332, Freeman: 0191 2231195
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Specialist Test:
Yes
Yes
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Specialist Contact Name:
Haematology and Haemostasis Healthcare Scientist
Haematology and Haemostasis Healthcare Scientist
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Specialist Telephone:
0191 213 9758
0191 213 9758
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Specialist Email: