Protocols - Hemorio

types 1 and 2 must, then, be monitored through FVIII:C dosage during the days prior childbirth and up to
two weeks later, due to fast decrease of FVIII and FVW levels in this period with hemorrhage risks. The
risk of bleeding is mild if the FVIII:C levels are superior to 40 IU/dl. When the levels are inferior to 20 IU/dl
and the patient is responsive to desmopressin, is medication can be administered at the moment of
childbirth and up to 2 days later, especially if the baby is delivered through a C-section . Desmopressin
can be safely used in pregnant women with VWD and hemophilia carriers, at any time of pregnancy and
as preparation for invasive procedures. A great surgical hemostasis is essential. In patients with VWD
type 3 it is recommended the infusion of factor concentrate during and after childbirth at 40 IU/kg dose,
with purpose to keep FVIII:C above 50% for 3-4 days. During pregnancy, in patients with VWD subtype
2B, the plateletopenia may worsen.
B – Pseudo-von Willebrand Disease
Also known as platelet type VWD, pseudo-von Willebrand Disease is NOT a type of VWD, once there is
no molecule defect of FVW. This condition is a platelet disease, arising from mutation with “gain of
function” at GPIB, which increases its affinity by multimeters of high molecular weight of FVW. The
heritage, of dominant autosomal character, has high penetrance and is very similar to subtype 2B of VWD.
The patients present delayed TS, limit levels of FVIII: C and FVW: Ag, low levels of FVW:RCo, absence of
multimeters of high molecular weight, RIPA hyper-aggregation with low doses of ristocetin and
plateletopenia. Bleeding must be treated with platelet transfusion and the use of DDVAP or FVIII: C/FVW
pellet is counter-indicated.
3- TREATMENT OF OTHER COAGULOPATHIES
Replacement therapy in less frequent inherited coagulopathies can be found in Table 6.
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