Abstract
We present a case of a 33-year-old woman in her third pregnancy diagnosed with platelet storage pool disorder who had previously suffered two postpartum major obstetric haemorrhages. Platelet storage pool disorder is a rare bleeding disorder where the platelet count is normal but platelet function is impaired due to deficiency of dense granules. A peripartum plan devised by an extensive multi-disciplinary team using principles for managing other bleeding and platelet function disorders helped minimise her risk of major haemorrhage. We also describe how point-of-care thromboelastography can help guide management and enable an individualised risk-benefit discussion with the woman about her anaesthetic choices.
Keywords: Pregnancy, platelet storage pool deficiency, thromboelastography, anaesthesia
Case report
A 33-year-old woman in her third pregnancy was referred to our hospital at eight weeks of gestation for antenatal care. Her first delivery had been vaginal but was complicated by a postpartum haemorrhage of 2 l. Subsequently, her sister developed significant bleeding during a routine dental procedure, and investigations revealed a familial platelet storage pool disorder in both women. Her second delivery was also complicated by a 2 l postpartum haemorrhage despite a planned elective caesarean section (ELCS) under general anaesthesia with pre-procedural desmopressin, tranexamic acid (TXA) and a platelet transfusion. Pre-pregnancy history included heavy menstrual bleeding, mild asthma and a single episode of supraventricular tachycardia.
In this pregnancy, her antenatal care was complicated by diagnosis of gestational diabetes at 32 weeks. Her multidisciplinary team peri-operative haemostatic management plan recommended: A peri-operative haemostatic management plan was constructed by her multidisciplinary team and included the following features.
Obstetric
An elective caesarean section was planned to ensure that delivery and any subsequent haemorrhage could be optimally managed. Routine oxytocin bolus at delivery was planned, followed by an oxytocin infusion and 1 g misoprostal rectally if uterine atony was present. Intramuscular ergometrine was to be avoided if possible because of the risk of haematoma, and carboprost was contra-indicated due to her asthma. Anti-thromboembolic stockings and intermittent calf compression until mobile.
Haematology
At 1-h pre-procedure, administration of 0.3 mcg/kg desmopressin followed by 1 g TXA intravenously and 50 mcg/kg recombinant factor VIIa at induction of anaesthesia was planned. These steps would be expected to provide adequate haemostasis for regional anaesthesia. After delivery, the second dose of recombinant factor VIIa (FVIIa) was to be given at 4 h, intravenous TXA six hourly for 24 h and the second dose of desmopressin 24 h after delivery if needed, followed by six weeks of oral TXA.
Anaesthetic
The obstetric anaesthetists were concerned about quantifying the risk of epidural haematoma formation in this woman. It was agreed that the decision on either a regional or a general anaesthetic technique should be discussed and agreed with the woman on the day of surgery by the anaesthetist performing the procedure.
She presented for ELCS at 39 weeks of gestation. Following administration of desmopressin, thromboelastography (TEG) (TEG 6, Hemostasis Analyzer System, Illinois, USA) was performed 30 min later (Figure 1) which showed a normal TEG trace. Following a risk-benefit discussion with the woman, it was agreed to administer the TXA and FVIIa before proceeding with ELCS under single-shot spinal anaesthetic. Following delivery of a healthy baby, good uterine tone was achieved with oxytocin bolus and infusion, without misoprostol. Measured blood loss was 800 ml. After transfer to the maternal recovery unit, the woman was administered the second dose of FVIIa and 24 h of intravenous followed by regular oral TXA. There were no complications prior to discharge.
Figure 1.
Pre-procedural TEG showing a normal trace.
CK: citrated kaolin; CRT: citrated rapid TEG; CKH: citrated kaolin heparinase; CFF: citrated functional fibrinogen.
Discussion
Platelet storage pool disorder is caused by quantitative and/or qualitative deficiency of dense granules. The characteristic diagnostic features are low-dense granule content of serotonin, nucleotides and calcium or decreased release of dense granule content, which leads to reduced platelet aggregation and a bleeding diathesis. Women are prone to heavy menstrual bleeding and significant bleeding following trauma, surgery and in the peripartum period. 1
Inherited platelet disorders as a group are increasingly recognised as diagnostic strategies improve. Whilst the true incidence is unknown, they are identified in approximately 30% of women referred to haematology with heavy menstrual bleeding. 2 Personalised peripartum management plans are prepared by multidisciplinary team for parturients by extrapolation using evidence and guidelines that exist for other bleeding disorders.3,4 Key principles include minimising the risk of bleeding and enhancing the woman’s own pro-coagulant mechanisms. When significant haemorrhage occurs, or is predicted, then, consideration should be given to platelet transfusion.
Use of TEG to guide decision-making
TEG assesses clot formation, strength and breakdown to give an overall picture of haemostasis. 5 Platelet dysfunction can be identified by a reduced maximum amplitude (MA) value; 6 however, this gives an overall impression rather than specific assessment of platelet-activation pathways. Whilst more specific platelet assessments such as platelet mapping are available, interpretation is difficult in the context of both an underlying platelet disorder and the hypercoagulable pregnant state, with a risk of obtaining misleading results. 7
Whilst utilisation of point-of-care TEG has gained traction in trauma and surgical specialties, it has yet to gain routine use in the peripartum period. Our hospital has expertise in use of TEG to guide perioperative blood management, and we have been exploring its use in the peripartum period. 6
In healthy women, antenatal TEG values are approximately normal values, with a gradual decrease in R-time (time until initial fibrin formation) and K-time (time to reach a clot strength of 20 mm), and an increase in MA (maximal amplitude) reflecting the hypercoaguable state of pregnancy and the immediate post-partum period.6,8 Platelet storage pool disorders cause a decrease in MA, reflecting reduced clot strength due to platelet dysfunction. Severe forms of platelet disorders may also cause abnormalities of other TEG values consistent with hypocoagulability. 9 Reassuringly, our woman’s TEG was normal, albeit following desmopressin administration, which would have increased platelet function and MA accordingly.
Whilst performing serial TEG studies would have allowed us to assess the efficacy of each step of our management strategy, we had to balance the half-life of each treatment with the time taken for the test. We chose to perform testing at 30 min following desmopressin administration as this would allow time for any improvement in platelet function to become apparent and gives enough time to obtain the MA value. A full test takes up to 1 h; however, the MA value is usually reported within 10–20 min. This allowed us to obtain a result and discuss management options within the time frame the haematologists had recommended to commence surgery. Additional tests would have been run if she developed a postpartum haemorrhage to guide both blood product replacement during the major haemorrhage and when to commence venous thromboembolism prophylaxis.
Regional vs. general anaesthesia
Given our primary aim in pre-procedural planning was to minimise our woman’s bleeding risk, regional anaesthesia was preferred over general because of its association with lower rate of haemorrhage and transfusion. 10 However, this has to be balanced against the potential increased risk of developing an epidural haematoma following regional anaesthesia, although the level of risk is difficult to quantify. 11 If she did develop an epidural haematoma, then neurosurgical decompression can be expected to be complicated and carry the risk of further morbidity because of the previously observed tendency for her to bleed with minimal tissue trauma.
As we anticipated that the haemostatic cover provided had corrected her bleeding risk, and her TEG (specifically the normal MA value) following desmopressin administration showed the mild hypercoagulable state of pregnancy, we believed that her risk of developing a spinal haematoma would be low. We discussed the risks and benefits with the woman and elected to proceed with spinal anaesthesia.
Differences between her caesarean sections
Despite her risk of major haemorrhage, this delivery proceeded uneventfully. There were two key differences between this and her previous delivery. First, our use of TEG enabled us to perform regional rather than general anaesthesia, reducing her bleeding risk. Second, our decision to use FVIIa in combination with desmopressin, rather than platelet transfusion, maximised the intrinsic function of her native platelets. Whilst there is little evidence to support using FVIIa over platelet transfusion, 12 its use is consistent with peripartum blood management strategies minimising transfusion risk.
Footnotes
Conclusion: This case involved the management of a multiparous woman with known platelet storage pool disorder who required a caesarean delivery. Our multidisciplinary team approach, combined with TEG to quantify her haemorrhage risk resulted in a management strategy with a successful outcome and little morbidity.
Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
Ethical approval: Not required
Informed consent: Written informed consent was obtained from the woman for her anonymised information to be published in this article.
Guarantor: TACS is the guarantor of the present work.
Contributorship: TACS initially drafted the article, all authors carried out final edits.
ORCID iDs: Timothy AC Snow https://orcid.org/0000-0002-8395-7857
Adrian England https://orcid.org/0000-0003-3872-3071
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