TY - JOUR
T1 - Flow cytometric mepacrine fluorescence can be used for the exclusion of platelet dense granule deficiency
AU - van Asten, Ivar
AU - Blaauwgeers, Maaike
AU - Granneman, Lianne
AU - Heijnen, Harry F. G.
AU - Kruip, Marieke J. H. A.
AU - Beckers, Erik A. M.
AU - Coppens, Michiel
AU - Eikenboom, Jeroen
AU - Tamminga, Rienk Y. J.
AU - Pasterkamp, Gerard
AU - Huisman, Albert
AU - van Galen, Karin P. M.
AU - Korporaal, Suzanne J. A.
AU - Schutgens, Roger E. G.
AU - Urbanus, Rolf T.
N1 - © 2019 International Society on Thrombosis and Haemostasis.
PY - 2020/3/1
Y1 - 2020/3/1
N2 - Background: δ-storage pool disease (δ-SPD) is a bleeding disorder characterized by a reduced number of platelet-dense granules. The diagnosis of δ-SPD depends on the measurement of platelet ADP content, but this test is time consuming and requires a relatively large blood volume. Flow cytometric analysis of platelet mepacrine uptake is a potential alternative, but this approach lacks validation, which precludes its use in a diagnostic setting. Objectives: To evaluate the performance of platelet mepacrine uptake as a diagnostic test for δ-SPD. Patients/Methods: Mepacrine fluorescence was determined with flow cytometry before and after platelet activation in 156 patients with a suspected platelet function disorder and compared with platelet ADP content as a reference test. Performance was analyzed with a receiver operating characteristic (ROC) curve. Results: Eleven of 156 patients had δ-SPD based on platelet ADP content. Mepacrine fluorescence was inferior to platelet ADP content in identifying patients with δ-SPD, but both mepacrine uptake (area under the ROC curve [AUC] 0.87) and mepacrine release after platelet activation (AUC 0.80) had good discriminative ability. In our tertiary reference center, mepacrine uptake showed high negative predicitive value (97%) with low positive predictive value (35%). Combined with a negative likelihood ratio of 0.1, these data indicate that mepacrine uptake can be used to exclude δ-SPD in patients with a bleeding tendency. Conclusion: Mepacrine fluorescence can be used as a screening tool to exclude δ-SPD in a large number of patients with a suspected platelet function disorder.
AB - Background: δ-storage pool disease (δ-SPD) is a bleeding disorder characterized by a reduced number of platelet-dense granules. The diagnosis of δ-SPD depends on the measurement of platelet ADP content, but this test is time consuming and requires a relatively large blood volume. Flow cytometric analysis of platelet mepacrine uptake is a potential alternative, but this approach lacks validation, which precludes its use in a diagnostic setting. Objectives: To evaluate the performance of platelet mepacrine uptake as a diagnostic test for δ-SPD. Patients/Methods: Mepacrine fluorescence was determined with flow cytometry before and after platelet activation in 156 patients with a suspected platelet function disorder and compared with platelet ADP content as a reference test. Performance was analyzed with a receiver operating characteristic (ROC) curve. Results: Eleven of 156 patients had δ-SPD based on platelet ADP content. Mepacrine fluorescence was inferior to platelet ADP content in identifying patients with δ-SPD, but both mepacrine uptake (area under the ROC curve [AUC] 0.87) and mepacrine release after platelet activation (AUC 0.80) had good discriminative ability. In our tertiary reference center, mepacrine uptake showed high negative predicitive value (97%) with low positive predictive value (35%). Combined with a negative likelihood ratio of 0.1, these data indicate that mepacrine uptake can be used to exclude δ-SPD in patients with a bleeding tendency. Conclusion: Mepacrine fluorescence can be used as a screening tool to exclude δ-SPD in a large number of patients with a suspected platelet function disorder.
KW - blood platelets
KW - flow cytometry
KW - platelet function testing
KW - platelet storage pool deficiency
KW - quinacrine
UR - http://www.scopus.com/inward/record.url?scp=85077394927&partnerID=8YFLogxK
U2 - https://doi.org/10.1111/jth.14698
DO - https://doi.org/10.1111/jth.14698
M3 - Article
C2 - 31815339
SN - 1538-7933
VL - 18
SP - 706
EP - 713
JO - Journal of thrombosis and haemostasis
JF - Journal of thrombosis and haemostasis
IS - 3
ER -