Rapid ADAMTS13 availability impacts treatment for microangiopathic hemolytic anemia and thrombocytopenia.
Thrombotic thrombocytopenic purpura (TTP) can present with a spectrum of clinical manifestations. When TTP is in a patient's clinical differential diagnosis, therapeutic plasma exchange (TPE) should be initiated emergently. Enzyme activity level of A Disintegrin And Metalloproteinase with a Thrombospondin type 1 motif, member 13 (ADAMTS13) in conjunction with the evolving clinical picture can guide further therapy, including duration and frequency of TPE and choice of fluid replacement. Our experience switching reference laboratories to obtain a more rapid turnaround time of ADAMTS13 activity level resulted in significant changes in clinical management, including fewer overall TPE procedures and the occasional use of albumin for a portion of the replacement fluid in patients without severe deficiency of ADAMTS13 and a low index of clinical suspicion for TTP. J. Clin. Apheresis 31:419-422, 2016. © 2015 Wiley Periodicals, Inc.
Department of Pathology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire. imarti17@jhmi.edu.
Isabella W
IW
Martin
Community Blood Services, Montvale, New Jersey.
Matthew C
MC
Katus
Department of Pathology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.
Christi-Lynn B
CL
Martin
Department of Pathology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.
Zbigniew M
ZM
Szczepiorkowski
Department of Pathology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.
James D
JD
Gorham
Department of Pathology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.
Nancy M
NM
Dunbar
D000071120:ADAMTS13 Protein / Q000097:blood*; D000743:Anemia, Hemolytic / Q000628:therapy*; D005440:Fluid Therapy; D006801:Humans; D010951:Plasma Exchange / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000628:therapy*; D012709:Serum Albumin / Q000627:therapeutic use; D013997:Time Factors
D012709:Serum Albumin; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
ADAMTS13; microangiopathic hemolytic anemia; therapeutic plasma exchange; thrombotic thrombocytopenic purpura; turnaround time