1. Hereditary Coagulopathy Mohammad reza baghaipour, MD Pediatrician, ISTH Fellow Comprehensive Hemophilia Care Center, Tehran, Iran

2. Bleeding disorders May be: Acquired Inherited Atuoimmune Drug
Thrombocytopenia
Platelet function disorders
Abnormal collagen
Clotting factor deficiencies
1 in 5000 to 1m

3. Menorrhagia
Menorrhagia most common symptom in women with bleeding disorders.( > 7days, > 80 ml )
vWD % (13 % - vWD)
BS 51%
GT 98%
Carriers 57%
F XI Def 59%
Rare BD 35-70%
Adolescent girls and perimenopause women

4. Menorrhagia
Assess hemoglobin content using the alkaline hematin method
Pictorial blood assessment chart (PBAC)

5. Menorrhagia
In most situations, practitioners must rely on menstrual history and clinical impression.
Variables that can predict a menstrual blood loss of more than 80 mL are:
Passage of clots greater than one inch (2.5 cm)
Low ferritin
The need for changing a pad or tampon more than hourly

6. Menorrhagia (Complete bleeding history):
Menorrhagia since menarche
Family history of a bleeding disorder
Personal history of one, but usually several, of:
*Epistaxis *Notable bruising without injury
*Minor wound bleeding *Bleeding in the oral or gastrointestinal tract
*Bleeding following dental extraction
*Unexpected post-surgical bleeding
*Recurrent midcycle pain due to ovulation bleeding
*Hemorrhage requiring blood transfusion
*Postpartum hemorrhage, especially secondary postpartum hemorrhage (after 24 hours).

7. Menorrhagia ( Lab test )
CBC
B/G
Ferritin level
PTT PT
vWF Ag / Act
FVIII levels
Platelet function test
Specific Factor Assay

8. Other Gynecological Conditions
Dysmenorrhea-
NSAID (should be avoided)
Acetaminophen
Hormonal Therapy
Hemorrhagic ovarian cysts
conservative management with the use of appropriate hemostatic agents
Endometriosis, fibroids (leiomyoma), polyps, or endometrial hyperplasia

9. Hemophilia Hemophilia is the commonest severe bleeding disorder
Hemophilia A (factor VIII deficiency)
Hemophilia B (factor IXdeficiency)
X- linked disorders
one in 5,000 men
Women are affected as carriers
Carriers may also have low factor levels
Experience significant bleeding symptoms

10. von Willebrand disease (VWD)
Most common inherited bleeding disorder
Autosomal disorders and equally affect women and men
Large epidemiological studies reported a % prevalence
Women are more likely to be symptomatic due to the bleeding challenges of menstruation and childbirth

12. Management of menorrhagia (adolescent )
Counseling prior to menarche
Preserving future fertility
Immunization

13. Pregnancy in Women with Bleeding Disorders
Preconception counselling:
Specially important for:
Women with severe bleeding disorders
potentially carry a severely affected baby
Benefits:
Adequate information
Available reproductive choices
Options for prenatal diagnosis
How and where to terminate the pregnancy
Immunization
Folic acid supplementation
Option for bleeding treatment (A DDAVP test dose )
Psychological support
Speak with a pediatric hematologist regarding the care of a potentially affected child.

14. Prenatal diagnosis (PND)
PND is primarily considered in carriers of hemophilia A or B
50% Affected child
50% Female carrier
Autosomal disorder
Sever forms
Consanguineous marriage
CVS ( w) 1% risk
Should receive prophylaxis prior to any invasive procedure
Fetal sex determination ( Mother blood 5-10w) (Sono 12w)
( if F, avoid testing, if M, avoid instrumental deliveries )
Pre-implantation genetic diagnosis (PGD) using IVF (overall live birth rate 22%)
Mother blood from 5 w to 10 w
Sono 12 w

15. Antenatal management Normal pregnancy: Carrier and affected female:
↑ Several coagulation factors including VIII, VWF, fibrinogen
↓ Fibrinolytic activity due to ↑plasminogen activator inhibitors
Carrier and affected female:
This rise is not enough and still risk of bleeding
Assay factor level at booking, 28w and 34w
women with deficiencies of fibrinogen or factor XIII :
↑ Miscarriage and placental abruption resulting in fetal loss or preterm delivery
Factor replacement is recommended
Approximately 20% of all pregnancies are complicated by at least one bleeding episode

16. Management of labor and delivery
Experienced Obstetrics
Hematologist with expertise in hemostasis
Laboratory
Pharmacy
Blood bank support

17. Management of labour and delivery
Mother with Bleeding tendency:
CBC, B/G, Factor level ( PT, PTT, PLT )
DDAVP ? ( safe for Mom & Inf, Water intoxication for OXY and Fluid )
Coagulation Factors are safe ( recombinant is preferred, PB19)
V/D versus C/S
Potentially affected fetuses:
Avoid Invasive intrapartum monitoring technique(fetal scalp electrode, fetal blood sampling)
Avoid instrumental deliveries (ventouse, midcavity or rotational forceps)
Normal vaginal delivery is not absolutely contraindicated (Avoid prolonged labour)
In situations where factor levels cannot be assessed, provided the f viii level is >50 IU dL in the third trimester, it is then
sufficient to assess the plt, PTT and PT .
Some Plasma derived has P B19 risk

18. Management of labour and delivery
Potentially affected fetuses:
Cesarean section may not completely eliminate the risk bleeding
Early recourse to cesarean section should be considered to minimize the risk
Cranial US If traumatic delivery
Factor Replacement if clinical signs suggestive bleeding in neonate
Intramuscular injections should be avoided ( oral Vit K, site Pressure)
Heel sticks should also have pressure applied for five minutes
Any surgical procedures (e.g. circumcision) should be delayed
Some factors (eg. vit K dependent F ) take time to get normal (6-12 mo)

22. Postpartum management
The most common causes of PPH:
Uterine atony, retained placenta, placenta pieces, and genital tract trauma
After the delivery, the elevated coagulation factors return to pre-pregnancy levels. ( Factor assay is necessary )
Therefore, the main risk of bleeding is after miscarriage or delivery
Primary PPH (blood loss of more than 500 mL in the first 24 hours after delivery)
Secondary PPH (excessive bleeding occurring between 24 hours and six weeks post delivery)
Perineal/vaginal hematoma are rare complications
Reducing the risk of PPH
prophylactic replacement therapy
three to four days for vaginal delivery
five to seven days for cesarean section

23. Postpartum management
Active management of the third stage of labor is very important
Local causes should be excluded even in women with bleeding dis.
Administration of prophylactic uterotonics
Early cord clamping
Controlled traction of the umbilical cord
Meticulous surgical hemostasis
Care to minimize maternal genital and perineal trauma
Oral tranexamic acid
Combined oral contraceptive pills
Close collaboration between hematologists, obstetricians, and anesthetists

24. The Take-home message Bleeding Disorders are not rare
Reduces quality of life
Not aware of their symptoms and do not seek medical advice
Lack of awareness among caregivers
Its treatment needs teamwork

25. Thank you for your attention
مرکز جامع درمان هموفیلی ایران پایین تر از میدان فاطمی تقاطع زرتشت و فلسطین