期刊
TRANSFUSION AND APHERESIS SCIENCE
卷 49, 期 3, 页码 644-646出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.transci.2013.07.005
关键词
Thrombotic microangiopathy; %-IPF; Therapeutic plasma exchange
类别
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. (C) 2013 Elsevier Ltd. All rights reserved.
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