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

International Journal of Hematology

Volumn 74, Issue 1, 2001, Pages 109-114

  Sasahara, Yoji ^a   Kumaki, Satoru ^a   Ohashi, Yoshiyuki ^a   Minegishi, Masayoshi ^b   Kano, Hirotsugu ^c   Bessho, Fumio ^c   Tsuchiya, Shigeru ^a  

^a TOHOKU UNIVERSITY   (Japan)

^b TOHOKU UNIVERSITY HOSPITAL   (Japan)

^c UNIVERSITY OF TOKYO   (Japan)

Author keywords

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

Indexed keywords

ADAM PROTEIN; ADAMTS13 PROTEIN, HUMAN; AUTOANTIBODY; BIOPOLYMER; METALLOPROTEINASE; VON WILLEBRAND FACTOR;

ACUTE KIDNEY FAILURE; ADULT; ARTICLE; AUTOIMMUNE DISEASE; BLEEDING DISORDER; BLOOD; BRAIN HEMORRHAGE; CASE REPORT; CHEMISTRY; CHILD; CHRONIC DISEASE; CLASSIFICATION; ENZYMOLOGY; FEMALE; GENETICS; HEMOLYTIC ANEMIA; HUMAN; IMMUNOLOGY; JAPAN; MALE; METABOLISM; MOLECULAR WEIGHT; PEDIGREE; PLASMA; RECESSIVE GENE; RECURRENT DISEASE; SYNDROME; THROMBOCYTE AGGREGATION; THROMBOCYTOPENIA; THROMBOTIC THROMBOCYTOPENIC PURPURA;

ADAM PROTEINS; ADULT; ANEMIA, HEMOLYTIC; AUTOANTIBODIES; AUTOIMMUNE DISEASES; BIOPOLYMERS; CEREBRAL HEMORRHAGE; CHILD; CHRONIC DISEASE; FEMALE; GENES, RECESSIVE; HEMORRHAGIC DISORDERS; HUMANS; JAPAN; KIDNEY FAILURE, ACUTE; MALE; METALLOENDOPEPTIDASES; MOLECULAR WEIGHT; PEDIGREE; PLASMA; PLATELET AGGREGATION; PURPURA, THROMBOTIC THROMBOCYTOPENIC; RECURRENCE; SYNDROME; THROMBOCYTOPENIA; VON WILLEBRAND FACTOR;

EID: 0035409473     PISSN: 09255710     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF02982559     Document Type: Article

References (2)

1. Upshaw JDJ. Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N Eng J Med. 1978;298:1350-1352.
2. Schulman I, Pierce M, Lukens A, Currimbhoy Z. Studies on thrombopoiesis. A factor in normal human plasma required for platelet production; chronic thrombocytopenia due to its deficiency. Blood. 1960;16:943-957.