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Clot waveform analysis using CS-2000i™ distinguishes between very low and absent levels of factor VIII activity in patients with severe haemophilia A.
Haemophilia. 2017 Sep; 23(5):e427-e435.H

Abstract

INTRODUCTION

A recently developed method to assess comprehensive coagulation function, clot waveform analysis (CWA), accurately detect low levels (<1 IU/dL) of factor VIII activity (FVIII:C) in haemophilia A patients (HA-pts). Improvements are needed, however, to differentiate patients with very low from absent levels of FVIII:C.

AIM

We attempted to optimize CWA using the coagulation analyser CS-2000i™ to distinguish between very low levels and absent FVIII:C in severe HA-pts.

METHODS AND RESULTS

Activated partial thrombin time (aPTT)-based clot waveforms were determined in FVIII-deficient plasmas mixed with various amounts of recombinant FVIII. Clot times (CT) were shortened, and maximum coagulation velocity (|min1|) and acceleration (|min2|) were increased in FVIII dose-dependently at levels ranging from 0.25 to 100 IU/dL. The lowest level of FVIII:C detected was 0.25 IU/dL. Plasma samples from modestly severe (MS-HA; 0.5-<1.0 IU/dL), very severe (VS-HA; 0.25-<0.5 IU/dL), extremely severe (ES-HA; <0.25 IU/dL) and inhibitor-positive HA-pts (HA-inh) were examined. The CT was markedly prolonged in all instances but showed significant differences between the different groups insufficiently. The |min1| and |min2| in HA-inh were lower compared to the other groups (P<.05). A new parameter (slope-|min1|) reflecting average coagulation acceleration was derived. This index (median) was lower in HA-inh (0.0042) compared to ES-HA (0.0068) and VS-HA (0.011) with greater significant differences (P<.01), and an index of <.005 reflected the total absence of FVIII in the presence of inhibitor.

CONCLUSION

The slope-|min1| parameter could provide a useful index for evaluating very low and absent levels of FVIII and/or the development of FVIII inhibitor in HA-pts.

Authors+Show Affiliations

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan. Course of Hemophilia Treatment & Pathology, Nara Medical University, Kashihara, Nara, Japan.Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.Sysmex Corporation, Kobe, Hyogo, Japan.Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.Sysmex Corporation, Kobe, Hyogo, Japan.Sysmex Corporation, Kobe, Hyogo, Japan.Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

28750470

Citation

Matsumoto, T, et al. "Clot Waveform Analysis Using CS-2000i™ Distinguishes Between Very Low and Absent Levels of Factor VIII Activity in Patients With Severe Haemophilia A." Haemophilia : the Official Journal of the World Federation of Hemophilia, vol. 23, no. 5, 2017, pp. e427-e435.
Matsumoto T, Nogami K, Tabuchi Y, et al. Clot waveform analysis using CS-2000i™ distinguishes between very low and absent levels of factor VIII activity in patients with severe haemophilia A. Haemophilia. 2017;23(5):e427-e435.
Matsumoto, T., Nogami, K., Tabuchi, Y., Yada, K., Ogiwara, K., Kurono, H., Arai, N., & Shima, M. (2017). Clot waveform analysis using CS-2000i™ distinguishes between very low and absent levels of factor VIII activity in patients with severe haemophilia A. Haemophilia : the Official Journal of the World Federation of Hemophilia, 23(5), e427-e435. https://doi.org/10.1111/hae.13266
Matsumoto T, et al. Clot Waveform Analysis Using CS-2000i™ Distinguishes Between Very Low and Absent Levels of Factor VIII Activity in Patients With Severe Haemophilia A. Haemophilia. 2017;23(5):e427-e435. PubMed PMID: 28750470.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clot waveform analysis using CS-2000i™ distinguishes between very low and absent levels of factor VIII activity in patients with severe haemophilia A. AU - Matsumoto,T, AU - Nogami,K, AU - Tabuchi,Y, AU - Yada,K, AU - Ogiwara,K, AU - Kurono,H, AU - Arai,N, AU - Shima,M, Y1 - 2017/07/27/ PY - 2017/04/10/accepted PY - 2017/7/28/pubmed PY - 2018/5/22/medline PY - 2017/7/28/entrez KW - clot waveform analysis KW - factor VIII KW - haemophilia KW - inhibitor SP - e427 EP - e435 JF - Haemophilia : the official journal of the World Federation of Hemophilia JO - Haemophilia VL - 23 IS - 5 N2 - INTRODUCTION: A recently developed method to assess comprehensive coagulation function, clot waveform analysis (CWA), accurately detect low levels (<1 IU/dL) of factor VIII activity (FVIII:C) in haemophilia A patients (HA-pts). Improvements are needed, however, to differentiate patients with very low from absent levels of FVIII:C. AIM: We attempted to optimize CWA using the coagulation analyser CS-2000i™ to distinguish between very low levels and absent FVIII:C in severe HA-pts. METHODS AND RESULTS: Activated partial thrombin time (aPTT)-based clot waveforms were determined in FVIII-deficient plasmas mixed with various amounts of recombinant FVIII. Clot times (CT) were shortened, and maximum coagulation velocity (|min1|) and acceleration (|min2|) were increased in FVIII dose-dependently at levels ranging from 0.25 to 100 IU/dL. The lowest level of FVIII:C detected was 0.25 IU/dL. Plasma samples from modestly severe (MS-HA; 0.5-<1.0 IU/dL), very severe (VS-HA; 0.25-<0.5 IU/dL), extremely severe (ES-HA; <0.25 IU/dL) and inhibitor-positive HA-pts (HA-inh) were examined. The CT was markedly prolonged in all instances but showed significant differences between the different groups insufficiently. The |min1| and |min2| in HA-inh were lower compared to the other groups (P<.05). A new parameter (slope-|min1|) reflecting average coagulation acceleration was derived. This index (median) was lower in HA-inh (0.0042) compared to ES-HA (0.0068) and VS-HA (0.011) with greater significant differences (P<.01), and an index of <.005 reflected the total absence of FVIII in the presence of inhibitor. CONCLUSION: The slope-|min1| parameter could provide a useful index for evaluating very low and absent levels of FVIII and/or the development of FVIII inhibitor in HA-pts. SN - 1365-2516 UR - https://www.unboundmedicine.com/medline/citation/28750470/Clot_waveform_analysis_using_CS_2000i™_distinguishes_between_very_low_and_absent_levels_of_factor_VIII_activity_in_patients_with_severe_haemophilia_A_ L2 - https://doi.org/10.1111/hae.13266 DB - PRIME DP - Unbound Medicine ER -