Juniper Publishers: Successful Management of Pregnancy and Cesarean Section in a Patient with a Qualitative Variant of Glanzmann Thrombasthenia
JUNIPER PUBLISHERS- JOURNAL OF GYNECOLOGY AND WOMEN’S
HEALTH
Journal of Gynecology and Women’s Health-Juniper
Publishers
Authored by Cécile Choux*
Short Communication
A 19-year-old woman with a qualitative variant of
Glanzmann Thrombasthenia (GT) was referred to our department for her
first pregnancy. The diagnosis of GT was revealed by epistaxis and
gingival bleeding. Her history of bleeding was limited to dental
procedures and haemorrhagic rupture of an ovarian cyst under platelet
transfusion. Her husband had normal platelet function tests and no
consanguinity was suspected. No anti-GPIIb-IIIa and anti-HLA antibodies
were detected before the pregnancy. No bleeding occurred during the
pregnancy. At 38 weeks, the membranes ruptured. A first concentrate of
platelets was transfused and labour was induced by ocytocin. It failed,
and a C-section was performed. A second concentrate of platelets was
administered just before the incision; recombinant activated factor VII
(rFVIIa) was available but not used. Post-partum haemorrhage (PPH) did
not occur.
Maternal haemoglobin was 11g/dL before and 8.6g/dL
after the C-section. Platelet count and ROTEM® test were monitored
daily. Two red blood cells and one platelet concentrate were prescribed
at day 1 and one platelet concentrate at days 3, 6 and 9 according to
the results. Tranexamic acid (TA) and iron were administered for six
weeks. No prophylaxis against thrombosis was used. The newborn did not
suffer from neonatal thrombocytopenia (platelet count=168 G/L at day 3).
GT is a rare autosomal recessive qualitative or
quantitative abnormality of the platelet glycoprotein complex GPIIb-III
a, which binds fibrinogen and is required for platelet aggregation.
Patients typically present severe mucocutaneous bleeding [1].
Diagnosis of quantitative GT is confirmed by flow cytometry exploration
of GpIIb-IIIa at the platelet surface, which could be used to follow
the efficacy of platelet transfusions. Our patient presented qualitative
GT, which is associated with less severe bleeding [1].
This is the first report of successful pregnancy and C-section in a
patient with a qualitative variant of GT. Currently, there is no
specific recommendation for the pregnancy follow-up of such patients.
Flow cytometry cannot be used in qualitative GT. Here, we monitored
platelet transfusions with ROTEM®.
Bleeding can be treated with anti-fibrinolytics
including TA or rFVIIa, and red-cell and platelet transfusions. The
latter can trigger all immunization leading to platelet refractoriness
and therapeutic failure. rFVIIa is expensive and is used only in cases
of refractoriness to platelet transfusions [2].
Given the high risk of bleeding in both mother and
foetus, peripartum management in GT is challenging. In cases of maternal
alloimmunization, transplacental passage of antibodies can induce
foetal thrombocytopenia of unpredictable severity and can cause in utero
death [3].
Intra partum bleeding was reported in 61% of GT cases. Platelet
alloimmunization was reported in 73% of pregnancies and associated with
four neonatal deaths [1].
No consensus exists concerning the management of
pregnancy in GT. Haemophilia centres and obstetric departments must work
in close collaboration to plan delivery, postpartum time and neonatal
care [2]. Regional anaesthesia is contraindicated [4]. Elective C-section is considered only for obstetric indications or a high risk of foetal bleeding [1,4]. Foetal scalp electrodes, scalp blood samples, ventouses or high forceps are contraindicated.
For PPH prophylaxis, two options are available for
vaginal delivery: 1- rFVIIa combined with TA, and HLA-selected platelets
if bleeding occurs despite this treatment; 2-platelet transfusion and
TA associated with rFVIIa only if antibodies are detected. For
C-section, platelets and TA are the first-line therapies with surgical
drains and regular haemoglobin tests to identify bleeding early. The
median time to secondary PPH is 10 days so TA should be maintained for
at least two weeks [4].
In our case, platelet transfusions were preferred to
rFVIIa because of the good response to platelets for dental procedures
and surgery, a low risk of alloimmunization because the defect was
qualitative and the cost of rFVIIa.
As an example of successful management of a C-section
in a variant GT patient, this case report highlights the challenges of
specific care for GT patients and the usefulness of ROTEM® to monitor
platelet transfusion.
Acknowledgment
We are grateful to “Centre de référence des pathologies plaquettaires” and Roselyne d’Oiron MD for monitoring the delivery.
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