TY - JOUR

T1 - Diagnosis of Thrombotic Thrombocytopenic Purpura by ADAMTS13 Activity Quantification

AU - Dimopoulos, Konstantinos

AU - Philips, Malou

AU - Goetze, Jens P.

N1 - Publisher Copyright: © American Association for Clinical Chemistry 2022.

PY - 2022

Y1 - 2022

N2 - BACKGROUND: Rapid quantification of ADAMTS13 activity in plasma is essential for establishing a diagnosis of thrombotic thrombocytopenic purpura (TTP); a rare, but potentially lethal disorder. The current methods for quantitating ADAMTS13 activity are manual and only available at specialized laboratories, which often results in initiation of specific treatments long before a diagnosis of TTP is established. METHODS: We compared the performance of the HemosIL, a novel and rapid automated method, and the current standard FRET (fluorescence resonance energy transfer) method in quantitating ADAMTS13 activity using 706 consecutive plasma samples collected over a period of 14 years. The clinical accuracy of both methods was further examined using 212 diagnostic samples. RESULTS: The correlation between the FRET and HemosIL methods in all 706 samples and in the 212 diagnostic samples was excellent (Pearson's r of 0.919 and 0.912, respectively). Both methods displayed a high degree of clinical accuracy using the current cutoff of ADAMTS13 activity <0.10 kIU/L (<10%) as diagnostic for TTP: the area under the curve (AUC) was 97.7% for the FRET method and 99.5% for the HemosIL method. When applying a lower cutoff (ADAMTS13 activity <0.05 kIU/L or <5%), the diagnostic accuracy of the HemosIL method increased further (AUC = 99.7%). CONCLUSIONS: A novel, rapid method for ADAMTS13 quantification is comparable to the more laborious FRET method in patients with possible TTP. A rapid analysis available in the acute setting assessing patients with possible TTP allows for improved care and optimized treatment of a life-threatening condition.

AB - BACKGROUND: Rapid quantification of ADAMTS13 activity in plasma is essential for establishing a diagnosis of thrombotic thrombocytopenic purpura (TTP); a rare, but potentially lethal disorder. The current methods for quantitating ADAMTS13 activity are manual and only available at specialized laboratories, which often results in initiation of specific treatments long before a diagnosis of TTP is established. METHODS: We compared the performance of the HemosIL, a novel and rapid automated method, and the current standard FRET (fluorescence resonance energy transfer) method in quantitating ADAMTS13 activity using 706 consecutive plasma samples collected over a period of 14 years. The clinical accuracy of both methods was further examined using 212 diagnostic samples. RESULTS: The correlation between the FRET and HemosIL methods in all 706 samples and in the 212 diagnostic samples was excellent (Pearson's r of 0.919 and 0.912, respectively). Both methods displayed a high degree of clinical accuracy using the current cutoff of ADAMTS13 activity <0.10 kIU/L (<10%) as diagnostic for TTP: the area under the curve (AUC) was 97.7% for the FRET method and 99.5% for the HemosIL method. When applying a lower cutoff (ADAMTS13 activity <0.05 kIU/L or <5%), the diagnostic accuracy of the HemosIL method increased further (AUC = 99.7%). CONCLUSIONS: A novel, rapid method for ADAMTS13 quantification is comparable to the more laborious FRET method in patients with possible TTP. A rapid analysis available in the acute setting assessing patients with possible TTP allows for improved care and optimized treatment of a life-threatening condition.

UR - http://www.scopus.com/inward/record.url?scp=85129997933&partnerID=8YFLogxK

U2 - 10.1093/jalm/jfab148

DO - 10.1093/jalm/jfab148

M3 - Journal article

C2 - 34977929

AN - SCOPUS:85129997933

VL - 7

SP - 637

EP - 649

JO - The journal of applied laboratory medicine

JF - The journal of applied laboratory medicine

SN - 2475-7241

IS - 3

ER -