Premium
Partial clinical improvement in Upshaw‐Schulman syndrome following prostacyclin infusion
Author(s) -
KONNO MUTSUKO,
YOSHIOKA AKIRA,
TAKASE TOSHIO,
IMAI TOSHIO
Publication year - 1995
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1995.tb03697.x
Subject(s) - medicine , microangiopathic hemolytic anemia , prostacyclin , von willebrand factor , pathogenesis , endocrinology , thrombotic thrombocytopenic purpura , gastroenterology , platelet
We report on a Japanese girl with Upshaw‐Schulman syndrome, a congenital disorder characterized by recurrent thrombocytopenia, microangiopathic hemolytic anemia, proteinuria and hematuria that can be transiently improved by the transfusion of plasma or various plasma components. Unusually Large von Willebrand Factor (ULvWF) multimers were found during both relapse and remission phases. Serial plasma levels of 6‐keto‐prostaglandin F 1 α (PGF 1 α), the stable metabolite of prostacyclin (PGI 2 ), were low at relapse. When the patient was treated with continuous PGI 2 infusion, the microangiopathic hemolytic process gradually subsided within 10 days. These results suggest that PGI 2 may be partly involved in the pathogenesis of this congenital disorder.