Factor VIII concentrate-responsive thrombocytopenia, hemolytic anemia, and nephropathy. Evidence that factor VIII:von Willebrand factor is involved in its pathogenesis.

@article{Hara1986FactorVC,
  title={Factor VIII concentrate-responsive thrombocytopenia, hemolytic anemia, and nephropathy. Evidence that factor VIII:von Willebrand factor is involved in its pathogenesis.},
  author={Takeshi Hara and Akihiko Kitano and Takashi Kajiwara and Takashi Kondo and Kohji Sakai and Youichirou Hamasaki},
  journal={The American journal of pediatric hematology/oncology},
  year={1986},
  volume={8 4},
  pages={324-8}
}
A 4-year-old Japanese girl had a congenital disorder that was characterized by recurrent thrombocytopenia, hemolytic anemia, hematuria, and proteinuria, which were repeatedly improved by the infusion of factor VIII concentrate. She developed the similar symptoms within 1 h after 1-desamino-8-D-arginine vasopressin (DDAVP) administration. Coagulation studies 30 and 60 min after DDAVP infusion showed a disappearance of large factor VIII:von Willebrand factor (VIII:vWF) multimers, which was the… CONTINUE READING

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