Publication

Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects

Blaauwgeers, M. W., Kruip, M. J. H. A., Beckers, E. A. M., Coppens, M., Eikenboom, J., van Galen, K. P. M., Tamminga, R. Y. J., Urbanus, R. T. & Schutgens, R. E. G., 14-Jul-2020, In : American Journal of Hematology. 95, 10, p. 1142-1147 6 p.

Research output: Contribution to journalArticleAcademicpeer-review

APA

Blaauwgeers, M. W., Kruip, M. J. H. A., Beckers, E. A. M., Coppens, M., Eikenboom, J., van Galen, K. P. M., Tamminga, R. Y. J., Urbanus, R. T., & Schutgens, R. E. G. (2020). Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects. American Journal of Hematology, 95(10), 1142-1147. https://doi.org/10.1002/ajh.25910

Author

Blaauwgeers, Maaike W. ; Kruip, Marieke J. H. A. ; Beckers, Erik A. M. ; Coppens, Michiel ; Eikenboom, Jeroen ; van Galen, Karin P. M. ; Tamminga, Rienk Y. J. ; Urbanus, Rolf T. ; Schutgens, Roger E. G. / Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects. In: American Journal of Hematology. 2020 ; Vol. 95, No. 10. pp. 1142-1147.

Harvard

Blaauwgeers, MW, Kruip, MJHA, Beckers, EAM, Coppens, M, Eikenboom, J, van Galen, KPM, Tamminga, RYJ, Urbanus, RT & Schutgens, REG 2020, 'Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects', American Journal of Hematology, vol. 95, no. 10, pp. 1142-1147. https://doi.org/10.1002/ajh.25910

Standard

Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects. / Blaauwgeers, Maaike W.; Kruip, Marieke J. H. A.; Beckers, Erik A. M.; Coppens, Michiel; Eikenboom, Jeroen; van Galen, Karin P. M.; Tamminga, Rienk Y. J.; Urbanus, Rolf T.; Schutgens, Roger E. G.

In: American Journal of Hematology, Vol. 95, No. 10, 14.07.2020, p. 1142-1147.

Research output: Contribution to journalArticleAcademicpeer-review

Vancouver

Blaauwgeers MW, Kruip MJHA, Beckers EAM, Coppens M, Eikenboom J, van Galen KPM et al. Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects. American Journal of Hematology. 2020 Jul 14;95(10):1142-1147. https://doi.org/10.1002/ajh.25910


BibTeX

@article{9c257aa35fb54d039881793fcd112f88,
title = "Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects",
abstract = "Phenotypic characterization of congenital platelet defects (CPDs) could help physicians recognize CPD subtypes and can inform on prognostic implications. We report the analyses of the bleeding phenotype and diagnostic characteristics of a large cohort of adult patients with a confirmed CPD. A total of 96 patients were analyzed and they were classified as Glanzmann thrombasthenia, Bernard-Soulier syndrome, dense granule deficiency, defects in the ADP or thromboxane A2 (TxA2) pathway, isolated thrombocytopenia or complex abnormalities. The median ISTH-BAT bleeding score was nine (IQR 5-13). Heavy menstrual bleeding (HMB) (80%), post-partum hemorrhage (74%), post-operative bleeds (64%) and post-dental extraction bleeds (57%) occurred most frequently. Rare bleeding symptoms were bleeds from the urinary tract (4%) and central nervous system (CNS) bleeds (2%). Domains with a large proportion of severe bleeds were CNS bleeding, HMB and post-dental extraction bleeding. Glanzmann thrombasthenia and female sex were associated with a more severe bleeding phenotype.",
keywords = "GUIDELINES, DISORDERS, SYMPTOMS, ADULT",
author = "Blaauwgeers, {Maaike W.} and Kruip, {Marieke J. H. A.} and Beckers, {Erik A. M.} and Michiel Coppens and Jeroen Eikenboom and {van Galen}, {Karin P. M.} and Tamminga, {Rienk Y. J.} and Urbanus, {Rolf T.} and Schutgens, {Roger E. G.}",
year = "2020",
month = jul,
day = "14",
doi = "10.1002/ajh.25910",
language = "English",
volume = "95",
pages = "1142--1147",
journal = "American Journal of Hematology",
issn = "0361-8609",
publisher = "Wiley",
number = "10",

}

RIS

TY - JOUR

T1 - Bleeding phenotype and diagnostic characterization of patients with congenital platelet defects

AU - Blaauwgeers, Maaike W.

AU - Kruip, Marieke J. H. A.

AU - Beckers, Erik A. M.

AU - Coppens, Michiel

AU - Eikenboom, Jeroen

AU - van Galen, Karin P. M.

AU - Tamminga, Rienk Y. J.

AU - Urbanus, Rolf T.

AU - Schutgens, Roger E. G.

PY - 2020/7/14

Y1 - 2020/7/14

N2 - Phenotypic characterization of congenital platelet defects (CPDs) could help physicians recognize CPD subtypes and can inform on prognostic implications. We report the analyses of the bleeding phenotype and diagnostic characteristics of a large cohort of adult patients with a confirmed CPD. A total of 96 patients were analyzed and they were classified as Glanzmann thrombasthenia, Bernard-Soulier syndrome, dense granule deficiency, defects in the ADP or thromboxane A2 (TxA2) pathway, isolated thrombocytopenia or complex abnormalities. The median ISTH-BAT bleeding score was nine (IQR 5-13). Heavy menstrual bleeding (HMB) (80%), post-partum hemorrhage (74%), post-operative bleeds (64%) and post-dental extraction bleeds (57%) occurred most frequently. Rare bleeding symptoms were bleeds from the urinary tract (4%) and central nervous system (CNS) bleeds (2%). Domains with a large proportion of severe bleeds were CNS bleeding, HMB and post-dental extraction bleeding. Glanzmann thrombasthenia and female sex were associated with a more severe bleeding phenotype.

AB - Phenotypic characterization of congenital platelet defects (CPDs) could help physicians recognize CPD subtypes and can inform on prognostic implications. We report the analyses of the bleeding phenotype and diagnostic characteristics of a large cohort of adult patients with a confirmed CPD. A total of 96 patients were analyzed and they were classified as Glanzmann thrombasthenia, Bernard-Soulier syndrome, dense granule deficiency, defects in the ADP or thromboxane A2 (TxA2) pathway, isolated thrombocytopenia or complex abnormalities. The median ISTH-BAT bleeding score was nine (IQR 5-13). Heavy menstrual bleeding (HMB) (80%), post-partum hemorrhage (74%), post-operative bleeds (64%) and post-dental extraction bleeds (57%) occurred most frequently. Rare bleeding symptoms were bleeds from the urinary tract (4%) and central nervous system (CNS) bleeds (2%). Domains with a large proportion of severe bleeds were CNS bleeding, HMB and post-dental extraction bleeding. Glanzmann thrombasthenia and female sex were associated with a more severe bleeding phenotype.

KW - GUIDELINES

KW - DISORDERS

KW - SYMPTOMS

KW - ADULT

U2 - 10.1002/ajh.25910

DO - 10.1002/ajh.25910

M3 - Article

VL - 95

SP - 1142

EP - 1147

JO - American Journal of Hematology

JF - American Journal of Hematology

SN - 0361-8609

IS - 10

ER -

ID: 131698642