Review of patients studied for coagulopathy in a haematology/oncology unit

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• 作者：I. Romero^a ; N. Conde^a ; ^{nuriaconde@hotmail.com" class="auth_mail" title="E-mail the corresponding author} ; D.G. Aldana^a ; A. Ruano^b ; A. Ferná ; ndez-Teijeiro^a
• 关键词：Paediatric bleeding ; Coagulopathy ; Von Willebrand disease ; Coagulation tests
• 刊名：Anales de Pediatría (English Edition)
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：84
• 期：2
• 页码：85-91
• 全文大小：460 K

文摘

Symptoms/signs suggestive of coagulopathy is a frequent complaint in Paediatric Haematology units. Both the clinical and family history are essential for diagnosis.

Patients and methods

Retrospective and descriptive study of patients referred to a Paediatric Haematology unit of a tertiary hospital for possible coagulopathy during 2012.

Results

A total of 47 children were studied, of whom 61.7% had not previously presented bleeding. The most frequent reason for referral was a prolonged activated partial thromboplastin time without any haemorrhage (42.5%), of these, 25% were diagnosed with coagulopathy with a real risk of bleeding. Patients referred due to a prolonged activated partial thromboplastin time with bleeding more frequently (41.7%) present coagulopathy with a real risk of bleeding. Children with a family history of bleeding are diagnosed more frequently with coagulopathy with real risk of bleeding: 37.5% (family history) vs. 14.3% (no family history). The most frequent diagnoses were: healthy children (48.9%), von Willebrand type 1 disease (19.1%), factor xii deficiency (19.1%), factor xi deficiency (4.2%), prekalikrein/high molecular weight kininogen deficiency (2.1%), acquired deficiency of factor x (2.1%), and factor ix deficiency (2.1%).

Conclusions

A thorough personal and family bleeding history and physical examination are the first steps for a correct differential diagnosis. The reason for referral should be based more on clinical bleeding and not just on an abnormal coagulation time. The most frequent diagnoses were type 1 von Willebrand disease and factor xii deficiency.