1) Hereditary bleeding disorders can cause health problems in women, especially related to their reproductive systems like menorrhagia, dysmenorrhea, and hemorrhagic ovarian cysts.
2) Menorrhagia, or prolonged/heavy menstrual bleeding, is common in women with von Willebrand disease, platelet disorders, and other bleeding disorders. It can be assessed and managed through hormonal therapy, hemostatic agents, blood transfusions, or surgery depending on the severity.
3) Preconception counseling is important to discuss issues like genetic implications, pregnancy and delivery risks, and options for prenatal screening or testing. Proper management can help women with bleeding disorders have healthy pregn
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Women with hbd
1. Health Problems in
Women with
Hereditary Bleeding
Disorders
Magdy El Ekiaby, MD
Shabrawishi HTC, Egypt
2.
3. Hereditary Bleeding Disorders
in Women
Hereditary platelet vWF vWF
F II F VIII
disorders TFPI F Xa F VIIIa
F IIa
F Xa TF F VIIa F Va F XI
vWD (1%) Platelet F V
TF bearing cell FV
F Va F XIa
Hemophilia A & B TF F
VIIa F X
carriers (<1/10,000) F IX F IXa
F Xa
F VIIIa
F XIa F Va
Coagulation factor Activated platelet
F II F IIa
deficiency I – XIII F IIa
F IIa
(1/0.5 – 2 million) F IIa
F IIa
F IIa
F IIa F IIa
F IIa F IIa
4. Inheritance
Hereditary bleeding diseases are inherited as recessive
characters
Women with heterozygote bleeding disorders usually
are asymptomatic, or experience mild bleeding
problems
In rare cases, there might be compounded
heterozygote inheritance of bleeding disorders in
which case bleeding symptoms may be severe
Women with homozygote inheritance pattern suffer
from severe bleeding symptoms, particularly those
related to their reproductive system, eg menstrual
5. Reproductive Health
Problems
Menorrhagea
Dysmenorrhea
Hemorrhagic ovarian
cyst
Pregnancy and delivery
Impact of bleeding
disorders on women
6. Menstrual Cycle
Normal menstrual cycle should be less than 7 days
long during which blood loss does not exceed 80 ml*
Women of modern societies may experience up to 500
cycles during their fertile age
Chronic disturbance of menses may have its impact on
the health, psychological and social life of the women
Every effort should be done to identify menorrhagea,
its probable causes, so that it can be properly
managed.
*ACOG, Management of anovulatory bleeding 2000, p.1-12
7. Menorrhagea
It is defined as prolonged menstruation (>7 days)
and/or excessive blood loss (>80 ml)
Excessive passage of blood clots, pain and flooding are
also common features of menorrhagea*
Adolescent girls and perimenopausal women may
suffer most, as the menstrual cycles are usually
anovulatory
*Kadir et al, Haemophilia 2009
8. Hemorrhagic Ovarian Cysts
In normal women, minor bleeding may occur with
rupture of the graafian follicle
Women with HBD are more likely to have more
significant bleeding at ovulation in the form of:
Hemorrhagic ovarian cyst
Broad ligament hematoma
Hemoperitoneum
Prevalence of hemorrhagic ovarian cysts in women
with HBD ranges from 2 – 25%*
*James, Hemophilia 2005
9. Clinical Assessment of
Menorrhagea
Iron deficiency anemia in women complaining of
menorrhagea is an indication of excessive blood loss
during menstruation
Iron deficiency anemia will not manifest for a long
time, while menorrhagea may be existing
Semi-quantitative assessment of menorrhagea can be
done using Pectorial Blood Assessment Chart
(PBAC)*
*Higham et al, Br J Obstet Gynaecol 1990
10. Assessment of menstrual blood loss using the
pictorial blood assessment chart (PBAC)
Scoring System SCORE:>100 = >80 ml blood loss
11. Prevalence of Menorrhagea in
Women with HBD
Disease Prevalence Reference
vWD 74-92% Kouides et al,
Hemophilia, 2000
Ragni et al,
Hemophilia, 1999
Berbnard-Soulier 51% Lopez et al, Blood 1998
Glanzmann Thromb. 98% George et al, Blood
1990
FXI deficiency 59% Kadir et al, American J
of Hematology 1999
Hemophilia Carrier 57% Kadir et al, Hemophilia
1999
RBD 35 – 70% James, Hemophilia
2005
12. Prevalence of HBD in Women with
Menorrhagea
Disease Prevalence reference
vWD 13% Shankar et al, Bjog 2004
Platelet dysfunction Limited data, up to 47% Philipp et al, J Thromb
& Haemost 2003
RBD Limited data
Menorrhagea should alert clinicians about possibility of an
existing bleeding disorder
13. When do we suspect HBD as a cause of
Menorrhagea?
Menorrhagea since Menarch
Recurrent midcycle pain due to ovulation bleeding
Family History of a bleeding disorder
Personal history of one or more of the bleeding
symptoms such as epistaxis, notable bruisis,
mucocutaneous bleeding, postoperative and
postpartum bleeding
Failure to respond to conventional management of
menorrhagea
16. Hormonal Therapy
Levonorgestrel IUS
(Mirena®) is the most
effective medical treatment
of menorrhagea, and is
useful in women with HBD
as well as a reversable
contraceptive tool
It is implanted for 3-5 years
It reduces endometrial
proliferation and reduces
blood loss during
menstruation
17. Hormonal Therapy
Combined hormonal therapy to reduce blood loss by
thinning the endometrium and probably increasing
FVIII & vWF levels, such as contraceptive pills,
transdermal contraceptive patches & vaginal rings
Oral progestogens
Gonadotropin-releasing hormone (GnRH) analogues
(stop ovulation)
19. Anti-fibrinolytic Agents
Tanexamic acid and Epsilon Amino Caproic Acid
(EACA), are known to have hemostatic effect and
may help to control menorrhagea
Oral Tranexamic acid (1g, 3 – 4 times daily) during
menstrual period is known to reduce menorrhagea
Usually it is well tolerated but side effects include
nausea, vomiting, headache and diarrhea
20. DDAVP
DDAVP (1-desamino-8-D-vasopressin), a synthetic
vasopressin that stimulates release of vWF and FVIII
from their endothelial stores
It has a formulation for treatment of nocturnal
enuresis and a hemostatic form
The hemostatic form comes as a nasal spray and
ampoules which are administered subcutaneously or
by iv infusion
For management of menorrhagea a daily dose of 150 –
300 micro-gram for a maximum of 3-4 days during
heaviest days of the cycle
21. DDAVP
It is mainly effective in Hemophilia A carriers, vWD
(except type 2B) and to some extent in Glanzmann
thrombasthenia and Bernard-Soulier Syndrome
Side effects include tachycardia, flushing, and
headache
Small risk of hyponatremia and water intoxication
with repeated doses, so better advise water intake
restriction during therapy
22. Acute Adolescent Menorrhagea
Usually achieved by a combination of hemostatic
agents and high doses of hormonal therapy
In severe cases specific factor concentrates and
intravenous anti-fibrinolytics may help
rFVIIa is successful in patients with severe platelet
dysfunction
Platelet transfusion (HLA matched whenever possible)
may be required in cases of severe thrombocytopenia
and thrombasthenia
24. Marriage
Women with bleeding
disorders usually have
social and psychological
problems connected
with marriage
Couples & their
relatives usually have
questions about sexual
life, hymen defloration,
risks of pregnancy and
delivery as well as
disease inheritance
25. Preconception
Advantages:
Provides adequate information on the genetic
implications of their disorders, the available
reproductive choices, and options for prenatal diagnosis
Allows planning for pregnancy and establishing how
and where pregnancy can be best managed
Immunization against HBV & HAV for those likely to
receive blood transfusion
DDAVP test dose to assess response
General hematenic supplmentation
26. Prenatal Diagnosis (PND)
Particularly important in hemophilia carrier due to the
severity of the disease in the male offspring
It is also of importance in heterozygote carriers in
cases of consanguineous marriage
PND diagnostic methods include:
Chorion Villus Sampling (CVS), weeks 11-14
1% risk of
Amniocentesis, weeks 15-20 abortion
Cordocentesis, weeks 18-20
Fetal sex determination
Pre-implantation diagnosis
27. Antenatal management
Increased levels of vWF, FVIII, Fibrinogen as well as
plasminogen activator inhibitors, particularly during
third trimester of pregnancy, reduces bleeding episodes
and complications in women with HBDs
(Bremme, Best Pract Res Clin Hematol, 2003)
20% of normal pregnancies may wetness a bleeding
episode due to obstetric problems, which should not
be overlooked in women with HBDs
Women with FI & FXIII deficiency are particularly at
risk of miscarriage, placental abruption and preterm
delivery which may require factor replacement during
pregnancy (Chi & Kadir,
28. Labour & delivery
Third trimester coagulation factor levels should be known before
delivery and factor replacement may be considered in those with
low levels
DDAVP may be used in hemophilia carriers and vWD during
delivery with caution
Matched packed RBCs should be reserved for emergency
bleeding
Cesarean section as well as other obstetrical procedures should be
evaluated for least traumatic manipulation of the fetus
Cord fetal blood sample to assess if the baby has inherited the
bleeding disorder
29. Postpartum management
Elevated coagulation and antifibrinolytic levels during
pregnancy, slowly return to its low levels in the
postpartum period
It is recommended to use prophylaxis of deficient
hemostatic agent immediately before delivery as well
as for 3-4 days after normal vaginal delivery and 5-7
days after cesarean section
14-21 days postpartum a secondary hemorrhage can
be expected
32. Quality of life
Women with HBD suffer many bleeding problems
during their fertile life
Many of these women experience anemia, fatigue
which affects their marital, familial, social and
practical life
They are at a higher risk of postpartum hemorrhage,
which may lead to severe morbidity and risk of
mortality
33. Conclusion
The prevalence of HBD is not insignificant
Women suffering from menorrhagea, particularly
since adolescence should be investigated to exclude
HBD
Women with HBD should receive care for their
reproductive life in specialized centers, or at least
under common supervision from an obstetrician and a
hematologist
Proper management of women with HBD can greatly
improve the quality of their lives