RT Book, Section
A1 Stowell, Sean R.
A1 Lollar, Pete
A2 Kaushansky, Kenneth
A2 Prchal, Josef T.
A2 Burns, Linda J.
A2 Lichtman, Marshall A.
A2 Levi, Marcel
A2 Linch, David C.
SR Print(0)
ID 1178755562
T1 Antibody-Mediated Coagulation Factor Deficiencies
T2 Williams Hematology, 10e
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260464122
LK hemonc.mhmedical.com/content.aspx?aid=1178755562
RD 2024/04/24
AB SUMMARYClinically  significant  autoantibodies  to  coagulation  factor  deficiencies  are  uncommon  but  can  produce  life-threatening  bleeding  episodes  and  death.  The  most  commonly  targeted  coagulation  factor  in  autoimmunity  is  factor  VIII.  Acquired  hemophilia  A,  which  results  from  these  antibodies,  can  be  either  idiopathic  or  associated  with  older  age,  other  autoimmune  disorders,  malignancy,  the  postpartum  period,  or  a  drug  reaction.  Treatment  options  to  prevent  or  stop  bleeding  in  acquired  hemophilia  A  include  factor  VIII–bypassing  agents,  anti-inhibitor  coagulant  complex  and  recombinant  factor  VIIa,  and  recombinant  porcine  factor  VIII.  The  underlying  autoimmune  disorder  frequently  responds  to  immunosuppressive  medication.  Antiprothrombin  antibodies  usually  are  found  in  patients  with  antiphospholipid  antibody  syndrome  and  can  be  associated  with  bleeding.  Antibodies  of  von  Willebrand  factor  are  found  in  patients  with  type  3  von  Willebrand  disease  in  response  to  infusion  of  von  Willebrand  factor.  Antibodies  to  factor  V  can  occur  as  autoantibodies  or  as  cross-reacting  anti-bovine  factor  V  antibodies  that  develop  after  exposure  to  bovine  thrombin  products  that  are  contaminated  with  factor  V.  Pathogenic  autoantibodies  also  have  been  described  that  target  thrombin,  factor  IX,  factor  XI,  factor  XIII,  protein  C,  protein  S  and  the  endothelial  cell  protein  C  receptor.