Thrombocytopaenia

Towards the end of pregnancy <5% of women have a

platelet count <150 109/l. This gestational thrombocytopaenia
carries no significance, but requires exclusion of
other disorders (Table 29.4). If the platelet count is <100 109/l, further investigations are
required (Table 29.5).

Immune thrombocytopaenic purpura

ITP results in thrombocytopaenia from autoantibodymediated

destruction of platelets. Such antibodies occur
idiopathically and also in association with other disorders
(Table 29.6). ITP mostcommonly presents as asymptomatic
maternal thrombocytopaenia, but transplacental
passage of antibodies can result in fetal thrombocytopaenia
in 915% and intracerebral haemorrhage in 1.5% of
babies with affected mothers. The diagnosis of ITP in
pregnancy is by exclusion of other disorders [33].
TREATMENT

Spontaneous bleeding is unlikely with platelets >20 109/l and monitoring of the patient and
platelet count

Haematological problems in pregnancy 275

Table 29.4 Thrombocytopaenia in pregnancy

Spurious (i.e. clumping or poor sampling)
Gestational
Immune thrombocytopaenic purpura (ITP)
Heparin-induced thrombocytopaenia (HIT)
Post-transfusion purpura (PTP)
Acute fatty Liver of pregnancy
Pre-eclampsia (PET)/ HELLP syndrome
Thrombotic thrombocytopaenic purpura (TTP)/ Haemolytic
uraemic syndrome (HUS)
Disseminated intravascular coagulation (DIC)
Drug induced thrombocytopenia
Systemic lupus erythematosis (SLE)/antiphospholipid
syndrome
Viral (HIV/EBV/CMV)
Congenital thrombocythemias/thrombocytopaenia
Hypersplenism
Type IIb vonWillebrands disease
Marrow dysfunction/haematinic deficiency
Table 29.5 Investigation of thrombocytopaenia
Blood film to exclude platelet clumps, MAHAor other
haematological disorders
Coagulation screen (to include fibrinogen and D-dimer levels)
Renal and liver function tests
Antiphospholipid antibodies
Anti-DNA antibodies to exclude SLE (antinuclear antibody is
sufficient as a screening test)
Table 29.6 Causes of ITP
Idiopathic
Helicobacter pylori
SLE
Lymphoma/chronic lymphocytic leukaemia
HIV
Drugs

are often all that is required, with the aim often attaining
an adequate platelet count for delivery.Aspontaneous
vaginal delivery or Caesarean Section can take place when
platelets are >50109/l. If the woman wishes or requires
epidural or spinal anaesthesia then a platelet count of
>80 109/l is recommended [33].
When required, treatment with either oral corticosteroids
or IVIgG produces 5070% response rates. The

IVIgG response usually lasts 23 weeks and repeated

dosing may be required. Secondary treatments include
high-dose methylprednisolone or azathioprine, or a combination
of these therapies with IVIgG. Other treatments
(vinca alkaloids and cyclophosphamide) are notsuit able
in pregnancy and splenectomy is also best avoided, but
Table 29.7 Causes of TTP/HUS
TTP Congenital
Pregnancy
Drugs (e.g. clopidogrel, ticlopidine, tacrolimus)
Combined contraceptive pill
Bone marrow transplant
SLE
Malignancy
HIV
E. coli-0157
HUS Pregnancy
Infection (cytotoxin producing E. coli or Shigella)
Drugs (e.g. cyclosporine, quinine, chemotherapy)

if essential, is bestcarried outbet ween 13 and 20 weeks

gestation.
ITP: THE MANAGEMENT OF DELIVERY

The babys plateletcountcannotbe reliably predicted

from any maternal features. Furthermore, fetal sampling
is hazardous or prone to spuriously low results.
Thus, procedures in labour and atdelivery thatpose an
additional bleeding risk should be avoided (fetal scalp
electrode, fetal blood sampling, ventouse and rotational
forceps). There is no evidence, however, that Caesarean
section is safer for the thrombocytopaenic fetus than an
uncomplicated vaginal delivery, as the nadir in platelets
is most often 2448 h after delivery. A cord blood platelet
countshould be determined in all babies and close monitoring
is required over the next 25 days.

Thrombotic thrombocytopaenic purpura/haemolytic

uraemic syndrome

Thrombotic thrombocytopaenic purpura (TTP) and

Haemolytic Uraemic Syndrome (HUS) are characterized
by thrombocytopaenia, microangiopathic haemolytic
anaemia (MAHA) and multiorgan failure. TTP is more
often associated with neurological abnormalities and nonrenal
organ ischaemia, while patients with HUS have
predominantly renal manifestations and usually occurs
post-partum. HUScan also be associated with haemolysis,
elevated liver, enzymes, low platelets (HELLP) syndrome.
However, there is a significant crossover and it is often difficult
to distinguish between the two [3436]. TTP occurs
most often as an idiopathic single episode, although there
is a congenital form that may recur. Like HUS, it may also
occur secondary to other influences (Table 29.7).
Von Willebrand factor is, on release from the endothelium,
cleaved by the metalloprotease,ADAMTS-13, resulting
in the correct balance of vWF multimers. TTP/HUS
is characterized by a failure of this cleavage. In TTP,

276 Chapter 29

this can be due to a congenital deficiency of ADAMTS13, but is more commonly due to an acquired autoantibody.
The resultant excess of circulating ultra-large
multimers leads to platelet aggregation and consumption,

leading to microvascular thrombosis. However, in

HUS, and in many cases of TTP, ADAMTS-13 is normal
and, indeed, a reduction in ADAMTS-13 is not specific
to TTP/HUS. Consequently, the exact mechanism is not
fully understood. However, the physiological coagulation
changes in pregnancy may predispose to the condition.
DIAGNOSIS

HUS typically presents post-partum with thrombocytopaenia,

haemolysis, and renal failure. While TTP is a
classic pentad of fever, haemolysis, thrombocytopaenia,
CNS signs and renal dysfunction, all five are only present
in around 50% of cases. TTP, particularly recurrent TTP,
usually presents before 24 weeks of pregnancy. Routine
blood clotting tests are often normal in the early stages
of TTP/HUS, but as the disease progresses there may be
coagulation activation and DIC.
TREATMENT

With the exception of endotoxin-related HUS (where supportive

care is the main requirement) and congenital TTP,
it is unlikely that a clear distinction between the two
syndromes will be possible in the majority of pregnancyrelated
cases. As a consequence, both are often considered
as a single syndrome when considering therapy, particularly
as there may be benefit in plasma exchange (PEX) in
non-toxin-related HUS [37].
The mainstay of treatment is PEX, which should be
instituted within 24 h of presentation and although the
optimal regime and fluid replacementis notcert ain, fresh
frozen plasma (FFP virally inactivated if possible) is the
common standard, although cryosupernatant may be preferred.
When exchange is notimmediat ely available, FFP
alone may be beneficial and, indeed, may be sufficientin
congenital disease. Intravenous methylprednisolone and
aspirin (when platelets >50 109/l) are often added to
PEX therapy. However, platelet transfusions should be
avoided in TTP. If the patient deteriorates, or does not
respond, higher volume, or frequency of exchanges, or
differentr eplacementfluid is recommended.

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