Background delta-storage pool disease (delta-SPD) is a bleeding disorder characterized by a reduced number of platelet-dense granules. The diagnosis of delta-SPD depends on the measurement of... Show moreBackground delta-storage pool disease (delta-SPD) is a bleeding disorder characterized by a reduced number of platelet-dense granules. The diagnosis of delta-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 delta-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 delta-SPD based on platelet ADP content. Mepacrine fluorescence was inferior to platelet ADP content in identifying patients with delta-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 delta-SPD in patients with a bleeding tendency.Conclusion Mepacrine fluorescence can be used as a screening tool to exclude delta-SPD in a large number of patients with a suspected platelet function disorder. Show less
Blaauwgeers, M.W.; Asten, I. van; Kruip, M.J.H.A.; Beckers, E.A.M.; Coppens, M.; Eikenboom, J.; ... ; Schutgens, R.E.G. 2019
