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CHAPTER 27  ■  Principles of Hemostasis and Thrombosis: Blood Coagulation Factors and Natural Coagulant Systems                                                         527




                        He  atic  ce  s  are  the    rinci  a   site  o   the  synthesis  o                                    Common Characteristics of Coagulation

                   coagu ation  actors. However, other ce  s, such as en  othe ia                                              Factors

                   ce  s, a so    ay an i    ortant ro e in the nor  a    rocess o

                   he  ostasis an   thro  bosis. C assica  y, the coagu ation  ac-                                             Proteins that are c otting  actors have  our characteristics in

                   tors have been   escribe   as reacting in a casca  ing sequence.                                            co    on. T ese characteristics are as  o  ows:

                   Mo  i  cations o  this sequence are now known to occur as                                                   1.  A   e  ciency o  the  actor genera  y   ro  uces a b ee  ing

                   the b oo    actors interact to  or   the   na  inso ub e ge ati-                                                 ten  ency   isor  er with the exce  tion o   actor XII,   reka -

                   nous thro  bus.                                                                                                   ikrein  (F etcher   actor),  an    high–  o ecu ar  weight


                                                                                                                                    kininogen (HMWK; Fitzgera     actor).
                   Basic Concepts of Blood Coagulation                                                                         2.  T e   hysica  an   che  ica  characteristics o  the  actor are


                                                                                                                                    known.
                   B oo   coagu ation is a sequentia    rocess o  che  ica  reac-                                              3.    he  synthesis  o   the   actor  is  in  e  en  ent  o   other

                   tions  invo ving     as  a    roteins,    hos  ho i  i  s,  an    ca -                                             roteins.

                   ciu   ions. Most o  the circu ating  actors (  ab e 27.1) that                                              4.  T e  actor can be assaye   in the  aboratory.

                    artici ate  in  the  coagu ation    rocess  are    esignate    by

                   Ro  an nu  era s. T e activate    or   o  an enzy  atic  ac-                                                       o   eve o   an un  erstan  ing o  the theory o  coagu ation

                   tor a    ears as a Ro  an nu  era   o  owe   by the su  x -a,                                               an   the un  er ying   rinci   es o  re ate    aboratory   roce-

                   whereas the inactive enzy  atic  actors, are in  icate   by the                                               ures, it is he    u  to co    are the characteristics (  ab e 27.2)

                   Ro  an nu  era  a one. For exa     e,  actor II,   rothro  -                                                o  various coagu ation  actors. T ree grou  s o   actors exist:

                   bin, is   esignate   as  actor II; however, in the active state,                                            the   brinogen grou  , the   rothro  bin grou  , an   the con-

                   it  is  IIa,  thro  bin.  Nonenzy  atic   actors  have  no  such                                            tact grou  .

                    esignations.  T e  Ro  an  nu  era    esignation   oes  not                                                     Te   brinogen group consists o   actors I, V, VIII, an   XIII.

                   in  icate the sequence o  reactions in the c otting   rocess.                                               T ese  actors are consu  e     uring the   rocess o  coagu a-

                   For exa     e,  actor X   rece  es  actor II in the coagu ation                                             tion. Factors V an   VIII are known to   ecrease   uring b oo

                    athway.                                                                                                    storage in vitro. T ese  actors are known to increase   uring








                       TABLE         27.1       Proteins in Blood Coagulation




                       Factor                                               Name                                                                          Alternate Terms




                       Coagulation Factors

                       I                                                    Fibrinogen


                       II                                                   Prothrombin


                       V                                                    Proaccelerin                                                                  Labile factor, Ac globulin

                       VII                                                  Proconvertin                                                                  Stabile factor, SPCA


                       VIII                                                 AHF                                                                           AHG, antihemophilic factor A


                       IX                                                   PTC                                                                           Christmas factor, antihemophilic factor B


                       X                                                    Stuart factor                                                                 Stuart-Prower factor

                       XI                                                   Plasma thromboplastin antecedent                                              PTA, antihemophilic factor C


                       XII                                                  Hageman factor                                                                Glass or contact factor


                       XIII                                                 Fibrin-stabilizing factor                                                     FSF


                       Others                                               Prekallikrein                                                                 Fletcher’s factor

                                                                            HMW kininogen                                                                 HMW kininogen, Fitzgerald’s factor


                                                                            vWF                                                                           Factor VIII–related antigen


                                                                            Fibronectin


                                                                            Antithrombin

                                                                            Heparin cofactor II


                                                                            Protein C


                                                                            Protein S


                       SPCA, serum prothrombin conversion accelerator; AHF, antihemophilic factor; AHG, antihemophilic globulin; PTC, plasma thromboplastin component; PTA,

                       plasma thromboplastin antecedent, FSF,   brin-stabilizing factor; HMW, high–molecular weight kininogen; vWF, von Willebrand factor.
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