This is my summary from the CME lecture.
Hope this helps. Please leave a message if you would like to discuss further.
Thanks
Acquired hemophilia
A. Pathophsiology
1. Autoantibody immune process :
against factor 8 - Type II kinetics – significantly decreased factor level.
Elderly patient >60yr c lympho prolifereative disorder (lymphoma, colon, lung cancer)
Immediate postpartum period women, underlying autoimmune process such as lupus, RA.
2. Alloantibody(congenital):
type I kinetic (factor level decreases in a predicted way)
B. Presentation
Bleeding problems such as GI bleed, or bruising, nasal or gum bleeding 20-30% mortality.
ER acute bleed into skin, mucus membrane, muscle, sometimes compartment syndrome.
No family hx of bleeding disorders. Important to obtain complete hx at time of presentation.
LABs
1. PTT elevated esp c pt of other autoimmune dz.
2. High ptt, should check factor 8
3. Lupus anticoagulation (risk for blood clot not bleeding cause)
R/o thrombocytopenia.
PTT mild high, still check factor 8 even if pt bleeds on gums.
Hx is the most important part.
Congenital disorder may have never been dxed before.
Any surgical procedure, teeth pulled in the past? Delivery, postpartum bleed?
Cold, flu, new med?
1/million
Congenital more common.
C. Treatment
Two arms
1. Control bleed using factor 8 inhibitor bypassing agent(feiba) activated prothrombin complex concentrate 80-100units/k q8-12hr or common factor 7a. AE is blood clot. Elderly pop who already may have CAD or stroke.
Reassess pt once stable. DIC may occur. Porcine factor 8 not available due to parvovirus. Maybe available in the future. Clinical judgement.
2. Irradicate inhibitor – have factor 8 level normal. Can be achived in acquired hemophilia. Irradication of antibody depends on how high antibody titer is. Low? Better response. Sometimes just can be steroids. Sugar can be out of control. High? 5-10? Combo of cytoxin and steroids(1mg/kg/d) together. 6 wks. Risk of bleeding. Mutaximab may be used in combo with steroid and cytoxin(cyclophosmide)
D. Many Adverse Effects.
Check renal def, blood glucose.
E. Follow up.
Check titer during the treatment to see the progress.
If after 4wks, no titer change?? Increase dose.
Be aware of it and think about it.
Anybody bleeding?? Check Platelet, CBC, PT/PTT
