Abstract
Introduction: Therapeutic guidance regarding delivery in factor V (FV)
deficient patients is scarce. Overall these patients are at increased risk of
postpartum hemorrhage (PPH), especially when FV levels are very low. The
preferred treatment in the peripartum period consists of administrating
fresh frozen plasma (FFP), targeting a FV level of 15–25 IU/dL.
Case report: We present the case of a 20-year old pregnant woman with
a FV activity <1 IU/dL, but very limited parahemophilia symptoms. She
was referred to our hospital because delivery of her first child had been
complicated by acute lung edema (ALE) following administration of FFP.
After the birth of her second child, she suffered from an important PPH,
despite adequate FV substitution. Administration of six additional units
of FFP resulted once more in ALE. Cardiologic work-up was warranted,
and revealed a non-compaction cardiomyopathy. Six years later, despite
negative advice regarding another pregnancy, this patient came again to
our hospital bearing a third child. This time, prior to delivery, she was
gradually given ten units of FFP together with diuretics, achieving a FV level
of 26 IU/dL. Acute lung edema and PPH did not develop.
Conclusions: When administering FFP, one should always remain aware
of fluid overload. In our case, symptoms became apparent more rapidly
because of a coexisting cardiomyopathy, but this does not need to be
the case in every patient. When parahemophilia patients get older, mild
preexisting heart failure may lead to similar situations when surgery with
a high bleeding risk is required
| Original language | English |
|---|---|
| Pages (from-to) | S82-S82 |
| Journal | Thrombosis Research |
| Volume | 135 |
| Publication status | Published - Feb 2015 |
| Event | 6th international symposium on Women's Health Issues in Thrombosis and Haemostasis - Berlin, Germany Duration: 13 Feb 2015 → 15 Feb 2015 |