Original Article

Korean J Hematol 2008; 43(4):

Published online December 31, 2008

https://doi.org/10.5045/kjh.2008.43.4.232

© The Korean Society of Hematology

만성 혈소판 감소를 보인 환아에서 발견된 폰 빌레브란드 병

박선민 이지혜 이건수

경북대학교 의과대학 소아과학교실

von Willebrand Disease in Childhood Chronic ITP

Sun Min Park, Ji Hye Lee, Kun Soo Lee

Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea

Abstract

Background: This study was performed to diagnose von Willebrand disease (vWD) in children with chronic immune thrombocytopenic purpura (ITP). Methods: Seventeen cases of chronic ITP were included in this study. Screening tests and specific tests were performed. These tests were also performed on their families when the patient was diagnosed vWD. Their past histories and family histories for bleeding tendencies were also reviewed. Results: Five cases were diagnosed with vWD: One had a lowlevel of vWF:RCo and factor VIII with a normal level of vWF:Ag, and others had a low level of vWF:RCo and vWF:Ag with a normal level of factor VIII. Among these, two cases showed abnormal screening test results, with prolongation of the aPTT or BT. The vWF multimer test could be performed in three cases, and two cases had a normal pattern and one had a abnormal pattern. Among the five vWD children, the past histories and family histories of a bleeding tendency could be obtained for four vWD patients and three families showed a bleeding tendency. But all the families were found to be normal on the first screening and the specific tests. Conclusion: von Willebrand disease was combined in 5 cases (29.4%) among the 17 chronic ITP children. More evaluations, such as the vWF multimer test and the ristocetin-induced platelet aggregation (RIPA) test are needed to confirm the subtype. Afollow-up test of the same type should be repeated on family members who have a history of bleeding, but they have normal test results for the diagnosis or exclusion of vWD

Keywords Chronic immune thrombocytopenic purpura, von Willebrand disease, Children

Article

Original Article

Korean J Hematol 2008; 43(4): 232-237

Published online December 31, 2008 https://doi.org/10.5045/kjh.2008.43.4.232

Copyright © The Korean Society of Hematology.

만성 혈소판 감소를 보인 환아에서 발견된 폰 빌레브란드 병

박선민 이지혜 이건수

경북대학교 의과대학 소아과학교실

von Willebrand Disease in Childhood Chronic ITP

Sun Min Park, Ji Hye Lee, Kun Soo Lee

Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea

Abstract

Background: This study was performed to diagnose von Willebrand disease (vWD) in children with chronic immune thrombocytopenic purpura (ITP). Methods: Seventeen cases of chronic ITP were included in this study. Screening tests and specific tests were performed. These tests were also performed on their families when the patient was diagnosed vWD. Their past histories and family histories for bleeding tendencies were also reviewed. Results: Five cases were diagnosed with vWD: One had a lowlevel of vWF:RCo and factor VIII with a normal level of vWF:Ag, and others had a low level of vWF:RCo and vWF:Ag with a normal level of factor VIII. Among these, two cases showed abnormal screening test results, with prolongation of the aPTT or BT. The vWF multimer test could be performed in three cases, and two cases had a normal pattern and one had a abnormal pattern. Among the five vWD children, the past histories and family histories of a bleeding tendency could be obtained for four vWD patients and three families showed a bleeding tendency. But all the families were found to be normal on the first screening and the specific tests. Conclusion: von Willebrand disease was combined in 5 cases (29.4%) among the 17 chronic ITP children. More evaluations, such as the vWF multimer test and the ristocetin-induced platelet aggregation (RIPA) test are needed to confirm the subtype. Afollow-up test of the same type should be repeated on family members who have a history of bleeding, but they have normal test results for the diagnosis or exclusion of vWD

Keywords: Chronic immune thrombocytopenic purpura, von Willebrand disease, Children

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