5. General management
• For the vast majority of cases the pregnancy and delivery should be treated as normal.
• In cases of moderate or severe thrombocytopenia an anaesthetic consultation is useful
to discuss analgesic options, since most units will not consider epidural anaesthesia
with platelet counts of less than 80.
• Where maternal platelet counts are low, similar management to that of maternal
immune thrombocytopenic purpura (ITP) is recommended, because of the small risk of
fetal thrombocytopenia.
• A trial of steroids should be considered when the count is 50–70.
• Counts should be monitored periodically. When maternal counts are less than 80 during
the pregnancy, a cord sample should be taken to ensure that the baby’s counts are
normal.
• Consider taking further neonatal samples on days 1 and 4, as neonatal
thrombocytopenia can present then.
• Hence, where possible, fetal scalp electrodes or sampling and high- or mid-cavity
operative delivery should be avoided. Caesarean section should be reserved for
obstetric indications only.3/24/20 elbohoty 5
6. •ITP only accounts for approximately 3% of these cases as
compared to gestational or incidental thrombocytopaenia of
pregnancy (74%) and hypertensive disorders of pregnancy
(21%)
IDIOPATHIC TROMBOCYTOPENIC PURPURA (ITP)
3/24/20 elbohoty 6
8. Diagnosis
•Careful history, examination and laboratory specimens are
helpful in excluding other causes of thrombocytopenia.
•A bone marrow test is not indicated unless there are unusual
features or lack of response to standard treatment.
•The main difficulty is differentiation from gestational
thrombocytopenia. However, this is not often a problem
clinically, since no treatment is required for either condition
when the platelet count is 70–80.
•It is unusual to have a count of less than 70 in gestational
thrombocytopenia.
3/24/20 elbohoty 8
9. Clinical Manifestation:
•Abnormally heavy menstruation
•Bleeding into the skin causes a characteristic skin rash
that looks like pinpoint red spots (petechial rash)
•Easy bruising
•Nosebleed or bleeding in the mouth
3/24/20 elbohoty 9
10. Diagnosis:
• Clinical
• The diagnosis of ITP in pregnancy remains one of exclusion as there
is no confirmatory laboratory test.
• Important to obtain a detailed history and physical examination to
exclude other secondary causes and to assess the clinical severity of
haemostatic defects.
• Laboratory
• The aim of investigation is to confirm thrombocytopenia and to
exclude secondary causes. If gestational thrombocytopenia is
suspected, only regular monitoring of platelet counts is required
without further investigations
3/24/20 elbohoty 10
11. Investigations:
• Full blood count
• Peripheral blood film: to exclude platelet clumping and red cell fragmentation
(in TTP, pre-eclampsia, HELLP or DIC)
• Coagulation screen (PT, APTT, BT, fibrinogen, D-dimer)
• Liver function tests
• HIV screening
• ANA
• Lupus anticoagulant/ anticardiolipin antibody for patients with past history of
unexplained pregnancy losses/ thrombosis
• Bone marrow examination is unnecessary unless there is suspicion of
myelodysplastic syndrome, leukaemia or lymphoma
3/24/20 elbohoty 11
12. Prepregnancy counselling
• ITP may relapse or worsen during pregnancy.
• If treatment of ITP is required it will carry both maternal and fetal risks.
• Around one-third of women will require treatment at some stage of pregnancy,
most commonly around the time of delivery.
• There is an increased risk of haemorrhage at delivery but the risk is small even if
the platelet count is low.
• Epidural anaesthesia may not be possible.
• Although it is not possible to predict accurately whether a neonate will be
affected, the risk is high if a sibling has had thrombocytopenia, or the mother has
undergone splenectomy.
• Maternal death or serious adverse outcomes for mothers with ITP are rare.
• The risk of intracranial haemorrhage for the fetus/neonate is very low.
3/24/20 elbohoty 12
14. Tips for Tapering Steroids
• Tapering must be individualized and patients must be observed for symptoms
• Parameters that must be taken into account when tapering glucocorticoids in
order to prevent adrenal crisis
Age
• Patients older than 40 must be weaned very slowly
• Duration of therapy
• No tapering needed for <1 wk of therapy
• Rapid tapering if 1-2 wk of therapy
• Slow taper if >2 wk of therapy
• Dosage of prednisone used: Taper rapidly to 40 mg/d
• Taper from 40-20 mg/d over several days
• Taper from 20 mg/d to none over an extended period of 2-4 wk, especially if
duration of therapy has been >2 w3/24/20 elbohoty 14
15. Management:
• Close collaboration between haematologist, obstetrician, neonatologist
and anaesthetist is needed to ensure a good pregnancy outcome.
• Platelet counts in women with ITP may decrease as pregnancy
progresses and need to be monitored closely as follows:
• 1st to 2nd trimester : monthly
• 3rd trimester : 2 weekly
• at term : weekly
• The decision to treat is based on assessment of the risk of significant
haemorrhage
• It is more often required to increase the count before delivery.
3/24/20 elbohoty 15
16. • Prednisolone is the usual first-line choice and it is often administered at lower
doses than those recommended for nonpregnant women to minimise the risk of
adverse effects on the mother (gestational diabetes, postpartum psychoses).
• A starting dose of 20 mg daily can be offered, escalating to 60 mg if no or an
inadequate response is seen after 1 week.
• Dosage should then be tapered to the minimum that is effective in maintaining
the count within the required range.
• Where the counts are very low, the woman is experiencing haemorrhage, or
there remains an inadequate response to steroids, intravenous immunoglobulin
should be considered, as it acts more quickly than steroids.
• Anti-D immunoglobulin appears to have efficacy equal to that of intravenous
immunoglobulin for women who are rhesus positive.
• Both these options are useful when a rapid increase in platelet count is required.
Other options are considered more rarely and include splenectomy and platelet
transfusion.
3/24/20 elbohoty 16
17. Modalities of treatment of ITP in pregnancy
• Corticosteroids and IVIG are effective and safe in pregnancy and are used as first line
therapy. (Grade B)
• Androgen analogs such as danazol and cytotoxic agents are contraindicated in the
treatment of ITP in pregnancy due to its teratogenecity.
• Splenectomy is considered only if above measures fail to elevate the platelet counts and
patient has serious bleeding. This is best deferred until the second trimester to prevent
miscarriage.
Recommendations : When to treat?
• Platelet count < 20 x 109/L before 36 weeks
• Symptomatic bleeding at any trimester
• Platelet count < 30 x 109/L after 36 weeks
3/24/20 elbohoty 17
18. • Antenatal platelets transfusion:
• Platelet count < 20 x 109/L before 36 weeks
• Symptomatic bleeding at any trimester
• The main concern at delivery for the mother is the risk of haemorrhage.
• Although there is no universally agreed safe platelet count, there is a general
consensus that a platelet count of at least 50 is safe for vaginal or operative
delivery.
• Where the maternal platelet count approaches 50, platelets should be available
on standby and management should be in close consultation with a
haematologist experienced in obstetric cases.
• Androgen analogs such as danazol and cytotoxic agents are contraindicated in the
treatment of ITP in pregnancy due to its teratogenecity.
• Splenectomy is considered only if above measures fail to elevate the platelet
counts and patient has serious bleeding. This is best deferred until the second
trimester to prevent miscarriage.
3/24/20 elbohoty 18
19. The accepted lower platelets level
Intervention Platelet count (109/l)
Antenatal, no invasive procedures 20
Planned Vaginal delivery 40
Operative or instrumental delivery 50
Epidural anaesthesia 80
3/24/20 elbohoty 19
20. Management during delivery:
• Platelet count above 50 x 109/L is safe for caesarian section under
general anaesthesia but not epidural anaesthesia.
• Epidural anaesthesia is best avoided because of the risk of epidural
haematoma and cord compression.
• However, patients who prefer epidural analgesia need to be admitted
earlier for IVIG infusion in order to raise the platelet counts to a safe
level >80 x 109/L.
• If platelet counts are less than 50 x 109/L and patient requires
immediate caesarian delivery, administer IVIG and
methylprednisolone.
• Give platelet transfusion just prior to surgery.
3/24/20 elbohoty 20
21. Neonatal considerations
• Since antibodies are of the IgG subtype, they can cross the placenta
and cause thrombocytopenia in the fetus and neonate.
• The main worry is possible intracranial haemorrhage in the neonate.
This is an extremely rare but devastating complication.
• The effect of the antibodies on fetal counts is unpredictable, maternal
treatments near term with steroids or intravenous immunoglobulin do
not have any effect on the fetal count and there is no correlation
between the severity of maternal thrombocytopenia and the fetal
count.
• The incidence of thrombocytopenia among neonates is reported as
between 14–37%.
• Approximately 5% of babies will have counts below 20 and a further
10% will have counts of 20–503/24/20 elbohoty 21
22. Gestational thrombocytopenia
• Diagnosis of exclusion; no tests are available to distinguish from immune
thrombocytopenic purpura
• Mild thrombocytopenia, platelet count usually >70 x 109/l
• No associated maternal bleeding
• No past history of thrombocytopenia outside pregnancy
• Occurrence in third trimester
• No associated fetal thrombocytopenia
• Spontaneous resolution after delivery
• May recur in subsequent pregnancies
3/24/20 elbohoty 22
23. Thrombotic thrombocytopenic purpura
• This is a rare, life-threatening disorder with a characteristic pentad of signs and
symptoms,
• which include microangiopathic haemolytic anaemia, thrombocytopenia,
neurological symptoms (varying from headache to coma), renal dysfunction and
fever. Often only some of these features are present.
• Thrombotic thrombocytopenic purpura occurs in about 1 in 25 000 pregnancies.
In addition to new cases, TTP (or haemolytic uraemic syndrome) that occurs
initially outside pregnancy may relapse during subsequent pregnancies.
• The time of onset in pregnancy is variable, ranging from the first trimester to
several weeks postpartum.
• 55% occurred in the second trimester. Maternal mortality was highest among the
newly presenting cases, particularly where pre-eclampsia was present.
3/24/20 elbohoty 23
24. Aetiology
• Thrombotic thrombocytopenic purpura has been shown to be due to a
severe deficiency of von Willebrand’s factor-cleaving protein (ADAMTS
13) which is a metaloproteinase
• This is most commonly an acquired deficiency caused by an
• autoantibody or, rarely,
• a congenital deficiency caused by a genetic defect.
• The two types can be distinguished by measurement of ADAMTS 13
antigen activity and inhibitor—inhibitor is absent in the congenital
form.
3/24/20 elbohoty 24
25. The lack of ADAMTS 13 leads to
• persistence of ultra-large multimers of von Willebrand’s factor
• that unfold and react with platelet receptors,
• resulting in microthrombi in many organs,
• particularly in the kidneys, brain and heart, and causing microangiopathic
haemolytic anaemia and thrombocytopenia.
3/24/20 elbohoty 25
26. • It usually undergoes rapid breakdown by the ADAMTS13 protein.
Where the ADAMTS13 protein is deficient, deposition of platelet-rich
thrombi occurs in the microcirculation leading to TTP.
• Levels of ADAMTS13 decrease in normal pregnancy during the second
and third trimester, and pregnancy-associated TTP manifests during
these trimesters or in the postpartum period.
3/24/20 elbohoty 26
27. Presentation (Pentad)
• Microangiopathic, hemolytic anemia
• Thrombocytopenia
• Neurologic abnormalities
• Confusion
• Headache
• Paresis
• Visual hallucinations
• Seizures
• Fever
• Renal dysfunction (AKI develops in 30–80% of cases of pregnancy- associated TTP,
which is a much higher rate than in TTP outside of pregnancy).
3/24/20 elbohoty 27
29. • Where thrombotic microangiopathy is considered as a potential diagnosis, specialist advice
should be sought promptly because treatment is complex and morbidity and mortality are
significant.
• TTP and HUS are managed with fresh frozen plasma infusion and/or plasma exchange. This will
replace the deficiency of normal anticoagulant factors and/or neutralise circulating antibodies.
• Plasma exchange in TTP has reduced maternal mortality from over 50% to less than
10%.However, microangiopathy of placental arterioles is hypothesised to contribute to a high
perinatal mortality rate of 30–80%.
• In addition, eculizumab is licensed for the treatment of atypical HUS. Eculizumab inhibits
activation of the complement pathway via an antiC5 blocking antibody.
• At present eculizumab is recommended for any patient with atypical HUS in whom the
ADAMTS13 activity is confirmed to be more than 10% pending further investigation, as it is
recognised that early treatment with eculizumab reduces morbidity.
• Data on eculizumab in pregnancy are limited but comparable biological agents have been used
without teratogenic effects and use in paroxysmal nocturnal haemoglobinuria is described in
pregnancy.
3/24/20 elbohoty 29
33. Surgical Tips for Performing a Cesarean Delivery on a
Patient with a Bleeding Diathesis
• Use a midline incision if there is clinically significant bleeding. Otherwise a transverse
incision is acceptable.
• Use electrocautery liberally, especially in opening the subcutaneous tissue.
• Close the uterus meticulously from the start. The more needle holes you put in the
uterus, the more it will bleed.
• Leave the bladder flap open to prevent hematoma formation that could later lead to
abscess. Cauterize the edge of the bladder flap if necessary.
• Close the peritoneum?! in order to prevent bleeding from the edges. This also prevents
subfascial bleeding from filling the peritoneal cavity and allows placement of subfascial
drains.
• If there is oozing, place subfascial drains and leave them in place until they stop
draining.
• Use skin staples, even in transverse incisions. This allows partial opening of the incision
if a subcutaneous hematoma or seroma forms.
• Place a pressure dressing over the incision and leave it in place until the danger of
bleeding subsides3/24/20 elbohoty 33
