Deficient activity of von willebrand factor-cleaving protease in patients with upshaw-schulman syndrome

Yoji Sasahara; Satoru Kumaki; Yoshiyuki Ohashi; Masayoshi Minegishi; Hirotsugu Kano; Fumio Bessho; Shigeru Tsuchiya

doi:10.1007/BF02982559

Deficient activity of von willebrand factor-cleaving protease in patients with upshaw-schulman syndrome

Yoji Sasahara, Satoru Kumaki, Yoshiyuki Ohashi, Masayoshi Minegishi, Hirotsugu Kano, Fumio Bessho, Shigeru Tsuchiya

MED - Medical Sciences

Research output: Contribution to journal › Article › peer-review

21 Citations (Scopus)

Abstract

We identified unusually large von Willebrand factor (vWF) multimers caused by deficient activity of vWF-cleaving protease in 2 patients with Upshaw-Schulman syndrome. The autoantibodies that inhibited the protease activity were not detected in the plasma of either patient. Periodic fresh-frozen plasma transfusion was effective for management of the hemolysis and thrombocytopenia. We detected enriched enzyme activity in a particular plasma fraction, although molecular cloning of this specific protease is needed to determine a more detailed pathogenesis and to develop new therapeutic approaches.

Original language	English
Pages (from-to)	109-114
Number of pages	6
Journal	International Journal of Hematology
Volume	74
Issue number	1
DOIs	https://doi.org/10.1007/BF02982559
Publication status	Published - 2001

Keywords

Chronic relapsing thrombotic thrombocytopenic purpura; von Willebrand factor multimer
Upshaw-Schulman syndrome
Von Willebrand factor-cleaving protease

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10.1007/BF02982559

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title = "Deficient activity of von willebrand factor-cleaving protease in patients with upshaw-schulman syndrome",

abstract = "We identified unusually large von Willebrand factor (vWF) multimers caused by deficient activity of vWF-cleaving protease in 2 patients with Upshaw-Schulman syndrome. The autoantibodies that inhibited the protease activity were not detected in the plasma of either patient. Periodic fresh-frozen plasma transfusion was effective for management of the hemolysis and thrombocytopenia. We detected enriched enzyme activity in a particular plasma fraction, although molecular cloning of this specific protease is needed to determine a more detailed pathogenesis and to develop new therapeutic approaches.",

keywords = "Chronic relapsing thrombotic thrombocytopenic purpura; von Willebrand factor multimer, Upshaw-Schulman syndrome, Von Willebrand factor-cleaving protease",

author = "Yoji Sasahara and Satoru Kumaki and Yoshiyuki Ohashi and Masayoshi Minegishi and Hirotsugu Kano and Fumio Bessho and Shigeru Tsuchiya",

note = "Funding Information: We are grateful to Dr. A. Wakisaka of the Japanese Red Cross Plasma Fractionation Center for providing plasma fractions. We are also grateful to Dr. T. Shichishima of Fukushima Medical College for the detection of CD55/ CD59-negative erythrocytes. Drs. H. Fujie, H. Asada, S. Kawai, and T. Hayashi at Tohoku University contributed to the clinical management of patient 1. This work was supported by grants from the Ministry of Education of Japan.",

year = "2001",

doi = "10.1007/BF02982559",

language = "English",

volume = "74",

pages = "109--114",

journal = "International Journal of Hematology",

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T1 - Deficient activity of von willebrand factor-cleaving protease in patients with upshaw-schulman syndrome

AU - Sasahara, Yoji

AU - Kumaki, Satoru

AU - Ohashi, Yoshiyuki

AU - Minegishi, Masayoshi

AU - Kano, Hirotsugu

AU - Bessho, Fumio

AU - Tsuchiya, Shigeru

N1 - Funding Information: We are grateful to Dr. A. Wakisaka of the Japanese Red Cross Plasma Fractionation Center for providing plasma fractions. We are also grateful to Dr. T. Shichishima of Fukushima Medical College for the detection of CD55/ CD59-negative erythrocytes. Drs. H. Fujie, H. Asada, S. Kawai, and T. Hayashi at Tohoku University contributed to the clinical management of patient 1. This work was supported by grants from the Ministry of Education of Japan.

PY - 2001

Y1 - 2001

N2 - We identified unusually large von Willebrand factor (vWF) multimers caused by deficient activity of vWF-cleaving protease in 2 patients with Upshaw-Schulman syndrome. The autoantibodies that inhibited the protease activity were not detected in the plasma of either patient. Periodic fresh-frozen plasma transfusion was effective for management of the hemolysis and thrombocytopenia. We detected enriched enzyme activity in a particular plasma fraction, although molecular cloning of this specific protease is needed to determine a more detailed pathogenesis and to develop new therapeutic approaches.

AB - We identified unusually large von Willebrand factor (vWF) multimers caused by deficient activity of vWF-cleaving protease in 2 patients with Upshaw-Schulman syndrome. The autoantibodies that inhibited the protease activity were not detected in the plasma of either patient. Periodic fresh-frozen plasma transfusion was effective for management of the hemolysis and thrombocytopenia. We detected enriched enzyme activity in a particular plasma fraction, although molecular cloning of this specific protease is needed to determine a more detailed pathogenesis and to develop new therapeutic approaches.

KW - Chronic relapsing thrombotic thrombocytopenic purpura; von Willebrand factor multimer

KW - Upshaw-Schulman syndrome

KW - Von Willebrand factor-cleaving protease

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Deficient activity of von willebrand factor-cleaving protease in patients with upshaw-schulman syndrome

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Keywords

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