RT Book, Section
A1 Zheng, X. Long
A1 Mustafa, Reem A.
A1 Mangalmurti, Nilam
A2 Schmidt, Gregory A.
A2 Kress, John P.
A2 Douglas, Ivor S.
SR Print(0)
ID 1201808112
T1 Thrombotic Thrombocytopenic Purpura, Hemolytic Uremic Syndrome, and Other Thrombotic Microangiopathies
T2 Hall, Schmidt and Wood’s Principles of Critical Care, 5<sup>th</sup> Edition
YR 2023
FD 2023
PB McGraw Hill
PP New York, NY
SN 9781264264353
LK accesssurgery.mhmedical.com/content.aspx?aid=1201808112
RD 2024/10/14
AB KEY  POINTSThrombotic  microangiopathy  (TMA)  describes  a  syndrome  of  microangiopathic  hemolytic  anemia  (MAHA)  and  thrombocytopenia  with  various  degrees  of  organ  dysfunction,  resulting  from  endothelial  injury  and  disseminated  microvascular  thromboses.Thrombotic  thrombocytopenic  purpura  (TTP)  may  be  caused  by  hereditary  or  acquired  (or  immune-mediated)  deficiency  of  plasma  ADAMTS13  activity,  while  hemolytic  uremic  syndrome  (HUS)  may  be  caused  by  Shiga  toxin-producing  Escherichia  coli  (STEC)  and/or  inherited  or  acquired  abnormalities  in  complement  activation  and  regulation  or  others.TTP  should  be  differentiated  from  HUS  and  other  related  TMA  resulting  from  malignant  hypertension,  disseminated  intravascular  coagulation,  collagen  vascular  diseases  with  vasculitis,  catastrophic  antiphospholipid  syndrome,  and  Hemolytic  anemia,  Elevated  Liver  enzymes,  and  Low  Platelets)  (HELLP)  syndrome,  etc.A  triple  therapy,  including  daily  therapeutic  plasma  exchange,  caplacizumab,  and  immunosuppressives  (e.g.,  corticosteroids  and  rituximab),  is  considered  the  standard  of  care  for  immune-mediated  TTP,  while  intermittent  plasma  infusion  may  be  sufficient  for  hereditary  TTP.Therapy  with  anti-C5  monoclonal  antibodies  (e.g.,  eculizumab  or  ravulizumab)  should  be  considered  for  atypical  or  complement-mediated  HUS.