Internal Medicine

0521779407-16 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:18

1116 Other Clotting Factor Deficiencies

■if surgery required, use estimated blood loss to estimate factor re-

placement

■discuss safest, least invasive approach for procedures with surgeons

specific therapy

Indications

■emergency replacement for trauma or spontaneous bleeding

■perioperative replacement for elective or emergency procedures

■prophylactic replacement to prevent bleeding

Treatment Options

■cryoprecipitate – replacement for factor I, XIII deficiency 6 bags 1200

mg fibrinogen for 80–100 mg level

■fresh frozen plasma – replacement for factor II, V, VII, X, XI, XIII

deficiency 5 units=5–15% factor level increment

■recombinant factor VIla – replacement for factor VII deficiency or

acquired anti-VIII inhibitor 90 mcg/kg q 2–3 hr=100% factor level,

then taper to q 4 hr; for anti-VIII inhibitor, once bleeding slows, may

switch to FEIBA, Autoplex

■FEIBA, Autoplex, Konyne (factor IX complex): factor II, V, X, XI 75–100

U/kg=100% level; then 50 U/kg q 8–24 hours

Side Effects & Contraindications

■FFP, cryoprecipitate – hepatitis, HIV, parvovirus, CJD, hives, hypo-

tension contraindicated if uncontrolled allergic reactions

■rFVIIa – thrombosis, phlebitis

■FEIBA, Autoplex, Konyne – thrombosis, hepatitis, inhibitor contra-

indicated if allergic reactions, thrombosis present

follow-up

During Treatment

■close monitoring for efficacy by blood loss, vital signs, and

hematocrit; potential neurologic, orthopedic, musculoskeletal com-

plications of continued bleeding: hypotension, neurologic sequelae

■assessment for complications of treatment:

➣FFP/cryoprecipitate – potential hepatitis A, B, C, HIV, parvovirus

➣FEIBA, Autoplex, Konyne – thrombosis, inhibitor, hepatitis

➣rFVIIa – thrombosis

Routine

■CBC, LFTs, inhibitor, HIV and hepatitis serologies q 6–12 months

■orthopedic, dental evaluation, review all bleeds & Rx q 6–12 month