Early-onset indicators of a hypercoagulable state and clinical complications in a cohort of children with sickle cell trait

被引：0

作者：

Reggiani, Giulia ^{[1
,5
]}

Boaro, Maria Paola ^{[1
]}

Menzato, Federica ^{[1
]}

De Bon, Emiliano ^{[2
]}

Bertomoro, Antonella ^{[3
]}

Casonato, Alessandra ^{[3
]}

Lucente, Fabrizio ^{[3
]}

Fortino, Cecilia ^{[3
]}

Sartori, Mariateresa ^{[3
]}

Steffan, Agostino ^{[4
]}

Martella, Maddalena ^{[1
]}

D'Agnolo, Mirco ^{[1
]}

Sainati, Laura ^{[1
]}

Colombatti, Raffaella ^{[1
]}

机构：

[1] Azienda Osped Univ Padova, Dept Womans & Childs Hlth, Pediat Hematol Oncol Unit, Padua, Italy

[2] Cittadellas Hosp, UOC Gen Med, Cittadella, Italy

[3] Azienda Osped Univ Padova, Dept Med, Internal Med Unit 1, Padua, Italy

[4] Ctr Riferimento Oncol, Dept Diagnost Lab & Cellular Therapy, Aviano, Italy

[5] Univ Padua, Dept Womans & Childs Hlth, Via Giustiniani 3, I-35128 Padua, Italy

来源：

PEDIATRIC BLOOD & CANCER | 2024年 / 71卷 / 06期

关键词：

ADAMTS13; children; coagulation; sickle cell disease; sickle cell trait; von Willebrand factor;

D O I：

10.1002/pbc.30971

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Adults with sickle cell trait (SCT) have a procoagulant state with increased risk of thromboembolism, but limited data are available for children. We compared the coagulation profile of children with SCT, different sickle cell disease (SCD) genotypes, and healthy controls. Compared to controls and similarly to HbSC patients, 41 SCT children (mean age 6.85 years; 20 males; 88% Africans) had a characteristic procoagulant profile: higher levels of factor VIII, von Willebrand factor (VWF) Ag and CBA, D-dimer; lower levels of ADAMTS 13 activity, ADAMTS13 activity: VWFAg, plasminogen activator inhibitor, tissue plasminogen activator. Moreover, 13/41 had clinical complications of SCD, five requiring hospitalization.

引用

页数：5

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