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1:1 000 000. If the clinical history is strongly suggestive of this
disorder then testing for abnormalities of the VWF/FVIII com-
Factor VII (FVII) deficiency
plex should be done on at least two separate occasions if initial
Factor VII is a vitamin K-dependent plasma glycoprotein that has results are inconclusive.
a half-life of approximately three to six hours.16 In the extrinsic Thrombocytopenia may accompany VW disease variant type
system, FVII interacts with tissue factor to accelerate the hydro- IIb and is thought to result from binding of the abnormal VWF to
lysis of FX, converting prothrombin to thrombin to form a per- platelets with subsequent platelet aggregate formation and clear-
ance.247 It may prove difficult to distinguish between the throm-
manent fibrin clot. Factor VII deficiency is inherited in an
autosomal recessive fashion and severe deficiency (FVII: bocytopenia of preeclampsia and variant VW disease.
C < 2 IU/dl) has a prevalence of approximately 1:300“500 000.239 Von Willebrand factor is synthesized in the endothelial cell and
as plasma levels of VWF rise during pregnancy248 many patients
Laboratory testing reveals a prolonged PT that corrects with 50:50
mix normal plasma, providing there is not an inhibitor. Activated achieve clinical and laboratory remission. For those patients who
partial thromboplastin time, TT, and fibrinogen levels are normal. require treatment, DDAVP at a dose of 0.3 mg/kg (maximum 20 mg
One should exclude vitamin K deficiency as a cause of bleeding. total) is the treatment of choice with the exception of patients with
VW disease types IIb and III.249 Infusion of DDAVP results in an
An FVII level less than 10% is indicative of homozygosity, but it
is important to know that bleeding does not necessarily correlate instantaneous release of VWF from the endothelium and an
with FVII level. In a study examining the surgical bleeding experi- immediate two- to three-fold rise in plasma levels for both
ence of individuals with FVII deficiency (FVII:C < 0.01 IU/ml) FVIII:C and VWF. However, repeated infusions can cause hypona-
who did not receive preoperative replacement therapy, the best tremia (occasionally resulting in seizures), as well as a tachyphy-
predictor for risk of bleeding was clinical data (history of sponta- laxis. Thus, in practice, DDAVP infusions are usually only repeated
neous joint bleeding), not laboratory values.240 once or twice at 12-hour intervals after the initial infusion. Patients
Acquired FVII deficiency can occur secondary to vitamin K defi- with type IIb VW disease are at risk for worsening of their throm-
ciency. This is usually associated with liver disease or vitamin K bocytopenia with infusion of DDAVP. For these patients and those
antagonists, such as warfarin. Treatment of bleeding in FVII defi- with type III (the most severe form of VW disease), the use of
cient individuals is generally with recombinant factor VIIa (rVIIa), Humate P (a viral inactivated plasma-derived product containing
both FVIII:C and active VWF) is the treatment of choice.247,249,250
although plasma and FIX and FVII concentrates have been



311
5 Other disorders


Factor VIII (FVIII) deficiency (hemophilia A) Factor IX (FIX) deficiency (hemophilia B,
Christmas disease)
This is an X-linked recessive trait, which results in deficiency
(activity level of 35% or less) of FVIII.230 Due to the X-linkage, This is an X-linked recessive bleeding disorder, which is indis-
affected females are very rare. Factor VIII is produced mainly in tinguishable from FVIII:C deficiency (hemophilia A) in clinical
the liver and is a cofactor that markedly enhances activated FIX spectrum and heredity. Fifty percent of the sons of heterozygous
on the surface of platelets. The clinical severity of bleeding corre- maternal carriers are hemizygous and affected; 100% of daugh-
lates with FVIII levels (see Table 17.5). ters of affected men and 50% of daughters of carrier mothers are
Due to lyonization (inactivation) of one of the X chromosomes heterozygous carriers. As is seen in hemophilia A, women may
in women, 10“30% of nonpregnant carriers may have low levels be significantly affected due to extreme lyonization. However,
of FVIII activity and are at risk for bleeding complications unlike hemophilia A, FIX levels do not rise during pregnancy so
(usually at surgery).230 Fortunately, FVIII:C increases in preg- pregnant carriers with low levels of FIX are at increased risk of
bleeding peripartum.255
nancy and, therefore, in the small number of carriers with symp-
tomatic disease, remission usually occurs in pregnancy.251 Five cases of FIX deficiency during pregnancy were reported
in the obstetric literature up to 1991.256 Four patients were fol-
However, if the levels remain low these patients are at risk of
hemorrhage. lowed throughout gestation with monthly FIX levels, and three
There are two reports of pregnancy in women with severe of four had intrapartum plasma or FIX prophylaxis. The fourth
FVIII deficiency.252,253 In one report, the woman (FVIII level patient presented at 28 weeks™ gestation with thrombocytopenia
<0.3 U/ml) received recombinant FVIII concentrate once or and a retrochorionic hemorrhage. She was treated with FIX and
twice weekly throughout her pregnancy.252 A continuous infusion eventually had a spontaneous vaginal delivery at 36 weeks™ gesta-
tion. No mention is made of the anesthetic management.256
of recombinant FVIII was administered throughout labor to
maintain the plasma FVIII level at normal levels. She received As FIX deficiency is an X-linked genetic disease, only 3% of
an epidural after coagulation was determined to be normal and the affected population are female. In a recent review of the
had a spontaneous uncomplicated vaginal delivery.252 In another obstetrical experience in women with FIX deficiency (two hemo-
case of hemophilia A (level <4%), the woman had a C/S for breech philia B and three hemophilia B carriers), Yang and Ragni noted
presentation.253 Preoperatively, her FVIII level was 6%. So she was that four of the five had excessive postpartum bleeding during
six of sixteen deliveries.257 It was more common in those preg-
treated with recombinant FVIII and had an uneventful cesarean
delivery under epidural anesthesia. After treatment, FVIII levels nancies where the women received fewer than four days of post-
were 198%. The recombinant FVIII infusion was maintained post- partum FIX replacement. There was no relationship between
operatively, but ten days after surgery she developed a brachial bleeding severity and the severity of FIX deficiency.
deep venous thrombosis.253 Although several cases of pregnancy in women with hemophi-
There are reports of bleeding in carriers of FVIII deficiency lia B have been reported anesthetic management is not men-
where the diagnosis was unknown. No information is given as to tioned. As FIX levels do not increase during pregnancy, women
whether these women received regional or general anesthesia.254 with low levels prior to pregnancy should be considered at risk
Acquired hemophilia can occur during pregnancy or postpartum for hemorrhage peripartum. Regional anesthesia is contraindi-
due to an acquired inhibitor of FVIII. These women often present cated in women with low levels of FIX unless replacement therapy
with severe bleeding. The aPTT is prolonged and is not corrected is given to normalize coagulation.
with addition of normal plasma. Remission usually occurs within
a few months.231 Treatment for severe bleeding is with FVIII
Factor X (FX) deficiency
concentrates (recombinant, monoclonal antibody purified pro-
ducts, or intermediate- and high-purity FVIII products).249
Factor X is a vitamin K-dependent clotting factor and is the first
enzyme in the common pathway of thrombus formation. It is
synthesized in the liver, has a relatively long plasma half-life
(40 hours), and is the most important activator of prothrombin.
Table 17.5 Severity of hemophilia based on factor activity
Severe FX deficiency (FX:C level <1 IU/dl) is inherited in
level
an autosomal recessive manner and is estimated to have a
prevalence of 1:1 000 000.258 The heterozygous form has a pre-
Clotting factor
Level Clinical severity activity valence of approximately 1:500 and heterozygous individuals
are usually asymptomatic although some do have a bleeding
Severe Spontaneous musculoskeletal <1
tendency particularly when challenged. Homozygous indivi-
and internal bleeding
duals usually have severe bleeding that may manifest as epis-
Moderate Occasional, spontaneous 1“5
taxis, hemarthrosis, or mucosal type bleeding. An individual
musculoskeletal bleeding
with a FX:C level of 1“5 IU/dl may bleed only following sur-
Mild Delayed onset bleeding after 5“35
gery or trauma. There is prolonged PT and aPTT, which will
trauma, surgery and dental
correct when mixed with normal plasma unless an inhibitor is
extraction
present.16



312
Chapter 17


In pregnancy, FX levels generally rise to 163% of normal deficiency was unknown prior to a PPH (FXI level 0.16 U/ml),
activity at 30 weeks, and return to normal six weeks postpartum. in the second woman, the aPTT prior to an urgent C/S was
Administration of FX may be needed in pregnant women with prolonged (FXI level 0.26 U/ml). She was given two units of
severe disease or who have had a previous severe outcome.16 FFP with correction of the aPTT and she received a combined
Usually FX replacement is unneccesary if the FX:C >10 IU/dl spinal“epidural anesthetic. The third woman was scheduled for
or if there is a lower level with no bleeding history. A multi- repeat C/S and an aPTT was prolonged (FXI level 0.39 U/ml). She
disciplinary approach is necessary to manage the pregnancy had an uneventful epidural for a previous C/S and so she had an
epidural anesthetic, which was also uneventful.266 In another
when both parents are known to have FX deficiency as there
would be a significant risk of bleeding for the mother and baby. report, a woman with FXI deficiency and an FXI inhibitor had
general anesthesia for a C/S.264
Few cases of FX deficiency in pregnancy have been
reported.258,259,260,261,262,263 In one, the FX levels did not increase
and the patient required FX concentrate due to placental abrup-
Factor XII (FXII) deficiency
tion.260 This patient eventually had an uneventful C/S with no
mention made of anesthesia. This is a rare disorder, which is transmitted in an autosomal
recessive manner. Mild deficiency is estimated to occur in
1.5“3.0% in Caucasians267 and some suggest that FXII deficiency
Factor XI (FXI) deficiency
is associated with thrombosis rather than bleeding. A recent
This deficiency is transmitted as an autosomal recessive trait and report examining the occurrence of thrombosis suggests that a
is reported to occur in between 0.1% and 0.53% of Ashkenazi combination of severe FXII deficiency (homozygous) with other
Jews.16 There also is an increased frequency in families of Italian risk factors, such as pregnancy, surgery, trauma, and other
and German background. The prevalence of severe deficiency thrombophilias, is more likely to be associated with thrombosis
than Factor XII deficiency alone.268 Factor XII deficiency does
(FXI:C level <10 IU/dl) is estimated at 1:1 000 000. Unlike the
classic hemophilias, the bleeding tendency does not correlate prolong the aPTT and has been associated with recurrent
abortion.267
well with FXI and assessment of the patient™s risk is best achieved
through a detailed history and family history. Spontaneous
hemorrhage is unusual and its occurrence may be dependent
Factor XIII (FXIII) deficiency
on other factor deficiencies. Factor XI deficiency has been asso-
ciated with Noonan syndrome, Gaucher syndrome, VW disease, The active form of FXIII is located mainly in platelets and mono-
FVIII deficiency, and FVII deficiency. These patients may present cytes. Activated FXIII crosslinks fibrin chains creating a stable
with a prolonged aPTT and normal PT. thrombus that is more resistant to fibrinolysis. During pregnancy
it is also present in the placenta.269 Factor XIII is made up of
Factor XI levels normally decrease during pregnancy, in con-
trast to other factors which increase. At 28 weeks, FXI level is 81% two subunits, A and S, with the former having fibrin-stabilizing
and at term, 62% of nonpregnant levels. Depending on the level activity. Factor XIII deficiency can be inherited in an autosomal
of FXI at term, these patients may require administration of the recessive manner or may be acquired. The frequency of defi-
specific factor. Occasionally, these patients develop an inhibitor ciency is 1:1 000 000“2 000 000 with consanguinity often a com-
that may require anti-inhibitor complex to correct coagulation.264 mon feature in affected families. Women with severe FXIII
Replacement factors are only used in women with a clinical deficiency (FXIII:C level <1 U/dl) are at risk of spontaneous
history of bleeding. bleeding, while those with levels of 1“4 U/dl may have moderate
Patients with severe deficiency usually bleed during surgery. to severe bleeding. Women with this deficiency have a history of
If the level is between 20 and 70 IU/dl, a bleeding history will severe bruising, muscle hematomas, hemarthroses, intracranial
indicate whether therapy is needed. Pregnancy carries the risk of hemorrhage, spontaneous pregnancy loss, PPH, and bleeding
PPH and bleeding at delivery and many advise administration after surgery or trauma. The PT and aPTT are normal.
of FXI concentrate to women with severe factor XI deficiency for During pregnancy, subunit S increases while subunit A tends to
C/S. In a report of women with severe FXI deficiency (<1 to 17 IU/ decrease causing a net reduction of FXIII that continues to
dl) there were 139 vaginal deliveries (51 women), 13 C/S (six decline. Although pregnancy loss is commonly reported, it is not
women), and five women had seven vaginal deliveries and five inevitable and those with severe FXIII deficiency should receive
C/S.265 The majority (43 women “ 70%) did not experience a PPH FXIII concentrate when pregnancy is confirmed. Treatment
should continue throughout pregnancy.270
during their 93 deliveries, and in those that had a PPH there was
no relationship to FXI level or genotype. The authors recommend
that FFP should be given only on demand in women with severe
Combined deficiencies
FXI deficiency during and after vaginal delivery, but they were
unable to make a similar recommendation regarding C/S.265 Combined FV and FVIII deficiency
Neuraxial anesthesia is contraindicated in women with FXI This is a rare autosomal disorder that is often seen in situations of
deficiency unless factor XI concentrate has been given with consanguinity. There are few data regarding the course in preg-
an adequate response. In one report of three women with FXI nancy. As FV does not consistently increase or decrease during
deficiency, all received neuraxial anesthesia.266 In one, FXI pregnancy while FVIII level will increase, bleeding will most likely



313
5 Other disorders


be dependent on the FV level. In one report of five unrelated DDAVP can be anticipated and may normalize the FVIII:C level.
Indian families there were no cases of pregnancy.271 This is safe therapy with none of the risks of factor concentrate.
Consideration for factor replacement is based on the factor levels
Vitamin K-dependent clotting factors inherited and their associated risk of delayed hemorrhage. These patients
deficiency (VKCFD, Borgschulte-Grigsby deficiency) should be identified early in pregnancy and a consultation with
There are few case reports of the autosomally recessive inher- an anesthesiologist and hematologist arranged so that manage-
ited combined deficiency of the vitamin K-dependent clotting ment can be discussed. General anesthesia for C/S is the techni-
factors. Individuals with this disorder experience a wide varia- que of choice if there is a coagulopathy.
tion in bleeding tendency with presentation at various ages.
There is a prolongation of PT and aPTT, which is dependent
Hypercoagulable states, heparin,
on the degree of reduction in activity of FII, FVII, FIX, and FX.
and anesthesia
Most individuals show some improvement with vitamin K ther-
apy. Occasionally factor replacement is necessary. There is a
Pregnancy is defined as a hypercoagulable state and parturients
single report of VKCFD during pregnancy that was managed
are therefore at greater risk of thrombosis.279 Parturients who
with oral vitamin K.272 Fresh frozen plasma was required for
have a hereditary predisposition to thrombosis (protein C, S, Z,
ongoing bleeding from an episiotomy.
or antithrombin III [ATIII] deficiency, or hereditary resistance to
activated protein C [Factor V Leiden or prothrombin gene muta-
Anesthetic management of patients with tion G2010A]) or those who have antiphospholipid antibodies
congenital coagulopathies (lupus anticoagulant, anticardiolipin antibody) are at even
greater risk.280 In addition to maternal risk there is increased
All patients with a diagnosis of a congenital coagulopathy benefit
risk of recurrent pregnancy loss, late fetal loss, preeclampsia,
from an early consultation with a hematologist and anesthesiol-
and abruptio placentae.279,280 Most anesthesiologists are familiar
ogist. This allows an assessment of specific factor levels, their
with most of the thrombophilias but may not be aware that
response during pregnancy, and anticipated management for
deficiency of protein Z, a cofactor for regulation of FXa activity,
labor and delivery. If there is any question about coagulopathy,
can also lead to pregnancy complications.279
regional anesthesia is contraindicated.
Many pregnant women considered at high risk of thromboem-
Patients with VW disease are frequently denied regional
bolic disease, or with known thrombophilia resulting in fetal risk,
anesthesia in spite of an improvement in their coagulation status
are now treated with heparin prophylaxis.279,281 Due to its lower
during pregnancy, due to an increase in antihemophilic factor
complication rate and stable and predictable pharmacokinetics,
and VWF. Many respond to the use of DDAVP.249 As VWF
LMWH may be the therapy of choice.279,282 However, because
decreases rapidly postpartum it will be necessary to continue to
of the potential risk of an epidural hematoma when regional
monitor their coagulation status and possibly administer DDAVP
anesthesia is administered concurrently with heparin, guidelines
in the postpartum period.
have been established as to the timing of regional anesthesia with
Regional anesthesia has been used successfully in patients with
respect to heparin (UFH, LMWH) administration.159 Every obste-
type I VW disease.273,274,275 For C/S, spinal anesthesia may be
trical anesthesiologist should be familiar with these guidelines
preferred due to the smaller size of needle. However, as the levels
and follow them.
of VWF decrease postpartum if epidural analgesia has been used,
one should remove the epidural catheter promptly following
Maternal hydrops
delivery. There is one report of uneventful regional anesthesia
in a parturient with type IIA disease following administration of
Humate P.276 Regional anesthesia was planned in a parturient Rarely, fetal and/or placental hydrops is accompanied by mater-
nal hydrops.283,284,285 This syndrome has variously been called
with type IIB disease, but her platelet count remained low follow-
ing administration of FVIII and platelets so general anesthesia Ballantyne syndrome, mirror syndrome (hydrops), triple edema,
was administered.277 and pseudotoxemia. Isoimmunization (Rh disease, anti-Kell,
Uniquely among the coagulation factors deficiency states, anti-Duffy antibodies), infection (various viruses such as parvo-
hemophilia A and B are inherited as X-linked recessive genes, virus), alpha thalassemia, sacrococcygeal teratoma, aneurysm of
but due to lyonization of the gene female carriers may exhibit the vein of Galen in the fetus, and placental tumors have been
coagulation abnormalities. Inwood and Meltzer278 have pointed implicated as the origin of hydrops in the fetus and placenta. The
out the potential anesthetic problems. Balance the benefits etiology of maternal hydrops is unknown.
versus risks of appropriate replacement therapy with the risks Clinically there are similarities to preeclampsia with massive
versus benefits of the anesthetic technique. Obviously, if there is edema, mild or moderate proteinuria, and mild hypertension,
an overt coagulopathy, regional anesthesia is contraindicated and hence the name pseudotoxemia. However, other well-documented
an alternative technique should be used for labor analgesia. cases have no proteinuria or hypertension. Generally the edema
Normalization of factor levels to minimize bleeding risk prior is severe, mainly involving the extremities. Clinically, the patient
to surgery will allow a broader spectrum of anesthetic options. may complain of shortness of breath due to pulmonary edema
In patients with a deficiency of FVIII:C, a good response to and/or polyhydramnios with upward pressure on the diaphragm



314
Chapter 17


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