RT Book, Section
A1 Long Zheng, X.
A1 Mangalmurti, Nilam
A2 Hall, Jesse B.
A2 Schmidt, Gregory A.
A2 Kress, John P.
SR Print(0)
ID 1107722755
T1 TTP, HUS, and Other Thrombotic Microangiopathies
T2 Principles of Critical Care, 4e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071738811
LK accesssurgery.mhmedical.com/content.aspx?aid=1107722755
RD 2024/04/23
AB Thrombotic  microangiopathy  (TMA),  a  pathologic  term,  describes  a  syndrome  of  microangiopathic  hemolytic  anemia  (MAHA)  and  thrombocytopenia  with  various  degrees  of  organ  dysfunction.TMA  includes  thrombotic  thrombocytopenic  purpura  (TTP)  and  hemolytic  uremic  syndromes  (HUS),  as  well  as  other  related  syndromes.TTP  is  primarily  caused  by  hereditary  or  acquired  deficiency  of  plasma  ADAMTS13,  whereas  HUS  is  mainly  caused  by  Shiga  toxin–producing  E  coli  and/or  abnormalities  of  complement  activation  and  regulation.TTP  should  be  differentiated  from  HUS,  disseminated  intravascular  coagulation  (DIC),  collagen  vascular  disease  with  vasculitis,  and  Hemolytic  anemia,  Elevated  Liver  enzymes,  and  Low  Platelets)  (HELLP)  syndrome,  etc.Plasma  therapy  remains  the  mainstay  of  therapy  for  TTP  and  atypical  HUS  (aHUS).  However,  the  treatment  for  aHUS  has  been  less  satisfactory.  Adjunctive  therapies  such  as  corticosteroids,  immunosuppressive  agents,  and  anti-CD20  monoclonal  antibodies  such  as  rituximab  or  anti-C5  monoclonal  antibodies  such  as  eculizumab  may  be  considered  for  refractory  TTP  or  HUS  cases.