Post Partum Hemorrhage in a Parturient with Twins and Platelet Storage Pool Disease
Abstract Number: 187
Abstract Type: Case Report/Case Series
Introduction: Platelet storage pool disease (PSPD) is a platelet aggregation disorder associated with bleeding diathesis. We present the management of a parturient with twins and PSPD complicated by post partum hemorrhage after spontaneous vaginal delivery (SVD).
Case: A 26 year old primigravida with twins was admitted secondary to preterm labor at 27 weeks gestation. She was placed on nifedipine tocolysis and betamethasone. PSPD was diagnosed at age 6 with a prolonged bleeding time (> 15 minutes) with abnormal platelet aggregation studies. Electron microscopy revealed platelets with diminished dense bodies. Four weeks later she had premature rupture of membranes. It was decided to attempt SVD with epidural analgesia. Platelet count, PT and PTT were normal. A thromboelastogram (TEG) was obtained which showed an R interval of 15.0 (normal 4-8) and angle 29.3 (normal 47-74). Prophylactic DDAVP (0.3mcg/kg) was administered. Repeat TEG showed R = 13.0, angle = 25.4. A unit of platelets was administered and repeat TEG showed R= 8.9, angle = 65.2. A labor epidural was placed and PCEA initiated. She became fully dilated and was taken to the operating room; epidural dosed with 10 ml of 2% lidocaine with 1:200,000 epinephrine. She had a twin SVD complicated by a second-degree vaginal and cervical laceration and uterine atony. She was treated with rectal misoprostol (1000 mcg), intramuscular hemabate (250 mcg), intramuscular methergine (200 mcg) and intravenous infusion of oxytocin (60 units). A second unit of pooled platelets and an additional dose of DDAVP were given. The lacerations were sutured and bleeding controlled. Estimated blood loss was 3500ml. Intraoperative hemoglobin decreased to 7.3 g/dl; two units of packed RBC were administered. She recovered in the ICU and discharged to the floor the next day.
Discussion: PSPD is a bleeding diathesis characterized by platelet aggregation abnormalities secondary to deficiency in one or more types of platelet granules. The most common form, dense storage pool disorder, is typically transmitted in an autosomal dominant pattern and is also associated with a number of hereditary disorders. It is associated with a decrease in the number and content of dense granules. Clinical manifestations vary from mild to severe bleeding. A prolonged bleeding time and aggregation abnormalities are classically seen, but some patients with a mild form may have a prolonged bleeding time but normal aggregation studies. Electron microscopy demonstrates absent or deficient alpha or dense granules, an ATP to ADP ratio greater than 3:1 in inactive platelets. PT and APTT are usually normal. TEG provides a useful tool for monitoring treatment response. Clinical management of PSPD is with prophylactic administration of DDAVP and platelet transfusions. The dose of DDAVP is 0.3 mcg/kg. The response to DDAVP is not entirely predictable, and platelet transfusions are often required.