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Immature platelet fraction can help adjust therapy in refractory thrombotic microangiopathic hemolytic anemia cases.

Abstract
Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura share presentations, therapies and diagnostic evaluation of activity of the metalloprotease ADAMTS13. Here, we report a patient with the clinical presentation of thrombotic microangiopathic thrombocytopenia, normal ADAMTS13, prolonged regimen of therapeutic plasma exchanges (TPEs), bone marrow biopsy showing adequate tri-lineage hematopoiesis, and low immature platelet fraction (%-IPF) (<1.0%). Low %-IPF suggested platelet hypoproduction; high steroid therapy, in conjunction with TPEs, resulted in the recovery of platelet count. Further investigation is needed to determine if %-IPF can guide therapy in cases of microangiopathic hemolytic anemias refractory to therapy.
AuthorsYelena E Kier, Lisa M Stempak, Robert W Maitta
JournalTransfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis (Transfus Apher Sci) Vol. 49 Issue 3 Pg. 644-6 (Dec 2013) ISSN: 1473-0502 [Print] England
PMID23899959 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright © 2013 Elsevier Ltd. All rights reserved.
Chemical References
  • ADAM Proteins
  • ADAMTS13 protein, human
Topics
  • ADAM Proteins (blood)
  • Anemia, Hemolytic (blood, diagnosis, therapy)
  • Blood Platelets (cytology)
  • Female
  • Humans
  • Middle Aged
  • Plasma Exchange
  • Thrombotic Microangiopathies (blood, diagnosis, therapy)

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