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Parturient with Delta Granule Platelet Storage Pool Disorder
Abstract Number: SUN-34
Abstract Type: Case Report/Case Series
An 18 y.o. G1P0, 40w0d parturient presented for obstetric anesthesia consultation for history of delta platelet storage pool disorder and hypermobility type Ehlers-Danlos syndrome. Significant bleeding with prior surgical procedures was documented. Hematology consultation and workup resulted in a diagnosis of “very severe deficiency” of delta granules.
Platelet storage pool disorder (PSPD) is a group of inherited defects in platelet granules associated with a wide spectrum of bleeding diathesis and reduced platelet aggregation (2). Granules are the small sack-like bodies inside platelets that contain adhesive proteins (alpha granules), or ADP, ATP, pyrophosphate, serotonin and calcium (delta granules) (4). Platelet activation causes release of the granular proteins, allowing further platelet recruitment and aggregation. In PSPD, the granules can be absent, reduced in number, contain an inappropriate ratio of the above compounds, or fail to release their contents into the bloodstream (2). Patients typically present with excessive bleeding in the setting of normal PT, aPTT, and platelet count (2). PSPD is diagnosed based on the following laboratory findings: decreased or absent dense granules on electron microscopy, prolonged bleeding time, abnormal platelet aggregation studies, and abnormal ADP/ATP ratio (4).
Few reports of safety with neuraxial anesthesia in PSPD patients exist. Hematology assessment of the severity of the patient’s PSPD is vital to plan for labor and delivery. Prophylactic administration of desmopressin and platelet transfusions are frequently required prior to a scheduled surgery or invasive procedure. Post operative antifibrinolytics are also used (2). In our patient, hematology recommended one unit of platelets prior to cesarean delivery, and transexamic acid 1300 mg po TID x 5-7 days postpartum.
Parturients diagnosed with PSPD create challenges for the obstetric anesthesiologist. Due to the small numbers of published case studies in patients with this disease, there exists an unknown risk of bleeding. Prominent bleeding can also be seen with Ehlers-Danlos Type 3 or hypermobility type (5). Our patient was scheduled by the obstetricians for induction of labor at 40w+0d. Remifentanil PCA was used for pain control during her labor contractions.
Cesarean delivery via general anesthesia was ultimately performed due non-reassuring fetal wellbeing. This parturient had an uncomplicated perioperative course and delivered a baby girl infant with APGAR scores of 9 and 9.
1. J Obstet Anesth, 15 (2006), pp. 7–12
2. Int J Obstet Anesth. 2011 Apr;20(2):173-7
3. Int J Obstet Anesth. 2011 Oct;20(4):360; author reply 361
4. Blood Reviews. Vol 29, Iss 3. Pages 153-162
5. British Jour of Haematology, 127, 491-500