Keywords : hemophilia, bleeding disorders, pregnancy
Hemophilias are inherited disorders associated with
decreased or absent coagulation factor VIII or IX with
an incidence of about in 1 in 10,0001 . The most common
variety is hemophilia A (85%), which is associated
with deficiency of factor VIII. Symptoms and signs
depend upon the degree of lack of coagulation factors
concerned.
A 25 year old gravida 2, para 1 with no living issue presented at 8 weeks of gestation for antenatal care. In her previous pregnancy, she had preterm intrauterine fetal death at eight months, ending in the delivery of a fresh stilI born female baby weighting 2 kgs. Puerperium was complicated by prolonged and heavy vaginal bleeding for 2 months. She was managed by a general practitioner with hemostatics and was not investigated for the cause of prolonged postpartum bleeding. Her previous medical, surgical and gynecological history was unremarkable.
Her general physical examination, obstetric examination
and routine antenatal investigations were normal.
During the third month, following a cut injury over her
right arm, she had continuous bleeding to the extent of
requiring five blood transfusions. Following the hematologist's opinion, coagulation screen was done
which revealed 53% of normal factor VIII activity. She
was continued on conservative management and the
pregnancy continued uneventfully. At 36 weeks, she
was advised admission in view of breech presentation,
but she refused. Next day she presented in emergency
in advanced labor with cervix was 8cm dilated and
delivered vaginally a female baby of 2.1 kg with Apgar
score of 7,8 and 9 at 1,5 and 10 minutes. One unit of
cryoprecipitate was given prophylactically following
which factor VIII levels rose to 58%. The post-partum
period was uneventful. Baby's factor VIII levels were
normal at birth.
Female carriers of hemophilia A usually do not have
clinical manifestations but cases where factor VIII levels
are unusually low (10-30%of normal), abnormal bleeding
may occur following traumatic injury or surgery. It is
suggested that, if the factor VIII levels continue to remain
low, cryoprecipitate should be given to cover delivery
and continued for 3-4 days postpartum to maintain
factor VIII levels greater than 50%. Factor VIII
concentrates should be avoided because they expose
women to hazards of multiple donations. Labor and
delivery should be carried out by the least traumatic
method",
