Page 2295 - Hematology_ Basic Principles and Practice ( PDFDrive )
P. 2295

Chapter 137  Rare Coagulation Factor Deficiencies  2037




                              Pregnancy: Suggested   Plasma Levels for   Invasive Procedures and   Delivery  Maintain at 100 mg/dL   throughout pregnancy  20%–30%  15%–25%  10%–20%  10%–20%  May withhold treatment   unless bleeding.   20%–40% if bleeding   occurs  250 units/wk through wk   22, then 500 units/  wk with a 1000 unit   bolus during labor








                                  Prophylaxis for Severe   Deficiency  Keep level 50 mg/dL   for recurrent   bleeders  40 factor IX units/kg   Q 5–6 days  Not usually required  20–30 µg/kg 2–3   times per wk  15–20 factor X   units/kg weekly.  40–50 Factor X units   twice weekly  Not required  40 factor XIII units/  kg q 28 days










                                Dosing Frequency   for Surgery or   Bleeding  Q 24–72 h   depending on   consumption  Q 48–72 h  Q 12–24 h  Q 2–12 h  Q 24 h  Q 1–2 days  Single dose may   be sufficient







                              Dose of Preferred   Treatment for   Surgery or   Bleeding  20–30 mg/kg  b  FFP 15–20 mL/  kg  Cryo 1 bag/10 kg  20–30 factor IX   units/kg  20 mL FFP/kg   then   5–10 mL/kg   q12 h  15–30 µg factor   VIIa/kg  PCC: 15–20   factor IX   units/kg  FX: 25 factor X   units/kg  15–20 factor XI   units/kg  20–30 factor XIII   units/kg







                                Minimum Plasma   Level Required for   Surgery  100 mg/dL until   healing   complete  30%  25%  15%–25%  25%–40%  30%–40%  20%–50%





                          Treatment Considerations for Rare Coagulation Factor Deficiencies.
                                Minimum Plasma   Level Required for   Hemostasis  >50 mg/dL  ~5%  ~10%  5%–10%  ~10%  15%  1%–2%







                                    Concentrate (Manufacturer)  RiaSTAP (CSL Behring) a  PCC a  None  NovoSeven, recombinant   Factor VIIa (Novo   Nordisk) a ;  PCC  PCC a ; Coagadex, Factor X   concentrate   (BioProducts   Laboratory)  Hemoleven (LFB   Biomedicaments);   Factor XI concentrate   (Bio Products   Laboratory)  Corifact a  (Formerly   Fibrogammin P, CSL   Behring);  Recombinant Factor XIII   (Tretten, Novo Nordisk) CRYO, Cryoprecipitate; FFP, fresh frozen plasma; PCC, pr











                                  Blood Product   Sources  CRYO FFP  FFP  FFP a  platelets  FFP  FFP  FFP a  CRYO FFP  a Preferred treatment for congenital deficiency b Assuming baseline fibrinogen is not known.







                         TABLE   137.3  Protein  Fibrinogen  Prothrombin  Factor V  Factor VII  Factor X  Factor XI  Factor XIII
   2290   2291   2292   2293   2294   2295   2296   2297   2298   2299   2300