Korean J Hematol 1994; 29(1):
Published online March 31, 1994
© The Korean Society of Hematology
Unusually Large vWF(ULvWF) multimer가 검출된 혈전성 혈소판감소성 자반증 1례
이미경, 김현태, 박애자, 채석래, 이상재, 이창훈, 송경순, 김현옥
중앙대학교 의과대학 임상병리과학교실,
중앙대학교 의과대학 내과학교실
A Case of Thrombotic Thrombocytopenic Purpura with ULvWF Multimer
We recently experienced a case of thrombotic thrombocytopenic purpura in a 34-year-old man with thrombocytopenia(46,000/μl), microangiopathic hemolytic anemia (Hb 6.6 g/dl), neurologic symptoms(dysarthria, coma) and fever. The large von Willebrand factor(vWF) multimeric forms(""Unusually Large"" vWF : ULvWF) were present in the patient's platelet but lack in platelet poor plasma during the acyte episode. After 12th
plasma exchange with fresh frozen plasma, the platelet count and vWF multimeric patterns became normal with much clinical improvement.
These findings support that the ULvWF multimers contribute to platelet aggregation in TTP.
Keywords TTP; "Unusually Large" vWF(ULvWF);
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Korean J Hematol 1994; 29(1): 111-116
Published online March 31, 1994
Copyright © The Korean Society of Hematology.
Unusually Large vWF(ULvWF) multimer가 검출된 혈전성 혈소판감소성 자반증 1례
이미경, 김현태, 박애자, 채석래, 이상재, 이창훈, 송경순, 김현옥
중앙대학교 의과대학 임상병리과학교실,
중앙대학교 의과대학 내과학교실
A Case of Thrombotic Thrombocytopenic Purpura with ULvWF Multimer
Mi Kyung Lee, Hyoun Tae Kim, Ae Ja Park, Seok Lae Chae, Sang Jae Lee, Chang Hun Lee, Kyung Soon Song, Hyun Ok Kim
Department of Clinical Pathology, Internal Medicine, College of Medicine, Chung Ang University, Seoul, Korea
Abstract
We recently experienced a case of thrombotic thrombocytopenic purpura in a 34-year-old man with thrombocytopenia(46,000/μl), microangiopathic hemolytic anemia (Hb 6.6 g/dl), neurologic symptoms(dysarthria, coma) and fever. The large von Willebrand factor(vWF) multimeric forms(""Unusually Large"" vWF : ULvWF) were present in the patient's platelet but lack in platelet poor plasma during the acyte episode. After 12th
plasma exchange with fresh frozen plasma, the platelet count and vWF multimeric patterns became normal with much clinical improvement.
These findings support that the ULvWF multimers contribute to platelet aggregation in TTP.
Keywords: TTP, "Unusually Large" vWF(ULvWF),
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