title: 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.
authors.affiliation: Departments of Pathology, University Hospitals Case Medical Center, Cleveland, OH, United States; Departments of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, United States.
authors.forename: Yelena E
authors.initials: YE
authors.lastname: Kier
authors.forename: Lisa M
authors.initials: LM
authors.lastname: Stempak
authors.forename: Robert W
authors.initials: RW
authors.lastname: Maitta
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000743:Anemia, Hemolytic / Q000097:blood / Q000175:diagnosis* / Q000628:therapy; D001792:Blood Platelets / Q000166:cytology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D010951:Plasma Exchange; D057049:Thrombotic Microangiopathies / Q000097:blood / Q000175:diagnosis* / Q000628:therapy
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: %-IPF; Therapeutic plasma exchange; Thrombotic microangiopathy