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///2018 Abstract Details
2018 Abstract Details2019-08-02T15:57:01-05:00

Thrombocytopenia of Known Origin? EDTA-Dependent Pseudothrombocytopenia in a Patient with Type 1 VWD and Pre-Eclampsia

Abstract Number: T1D-7
Abstract Type: Case Report/Case Series

Jessica Rock MD1 ; Cathy Drexler MD2; Sarah Hall MD3

Case:

A 24-year-old G1P0 with history of Type 1 von Willebrand’s Disease (VWD) presented with severe pre-eclampsia at 33 weeks gestation. The obstetric team proceeded with IOL due to difficulty with blood pressure control. Although she had a remote history of thrombocytopenia, platelet count at 14 weeks gestation was 162K. During admission initial platelet count was 67K, with platelet “clumping” reported. Repeat sampling in a citrate tube resulted in platelet count of 200K, consistent with EDTA-dependent pseudothrombocytopenia (EDTA-PTCP). As VWF activity levels were >50 IU/dL, hematology recommended Humate-P 40 U/kg only in the event of bleeding.

An epidural was placed for labor analgesia. Ultimately, she underwent CD with epidural anesthesia for failure to progress. CD was notable for excessive bleeding prior to uterine incision, treated with Humate-P 3712 units IV. After achieving hemostasis, an infant was delivered with Apgars 4, 8, and 8. Postpartum course was uneventful.

Discussion:

EDTA-PTCP is a low platelet count reported by an automated analyzer, usually because of clumping due to antibodies that react with platelet antigens exposed by EDTA. It is a cold agglutinin reaction only found in vitro. Maternal IgG can cross the placenta, leading to transient neonatal PTCP. No hemorrhagic tendencies exist because the actual platelet count is normal. Failure to recognize the phenomenon in the parturient or neonate can lead to unnecessary additional testing or treatments. Also, failed recognition in the parturient may lead to inappropriate changes in management. The process could have been missed in this patient due to her remote history of thrombocytopenia or pre-eclampsia; if the value had simply been accepted, she may not have received neuraxial and would have had a general anesthetic for her CD. Under different clinical circumstances, her obstetric diagnosis and management could also have been changed.

Type 1 VWD accounts for 80% of VWD. Due to variable penetrance and phenotype, it can be difficult to support the diagnosis with genealogy. Levels of VWF and FVIII rise during late pregnancy, often correcting to normal range. Recommendations include checking FVIII and VWF during the third trimester. Although evidence to guide clinicians based on levels is lacking, the usual threshold for treatment with DDAVP or factor replacement is 50 IU/dL. Excessive bleeding is treated regardless of factor levels. Because VWF levels fall again over 4-6 weeks postpartum, patients are at risk for delayed PPH.

1. Korterink JJ, Boersma B, Schoorl M, Porcelijn L, Bartels PC. Pseudothrombocytopenia in a neonate due to mother? Eur J Pediatr. 2013;172(7):987-989.

2. Xiao Y, Yu S, Xu Y. The Prevalence and Biochemical Profiles of EDTA-Dependent Pseudothrombocytopenia in a Generally Healthy Population. Acta Haematol. 2015;134(3):177-180.

3. Lipe BC, Dumas MA, Ornstein DL. Von Willebrand disease in pregnancy. Hematol Oncol Clin North Am. 2011;25(2):335-358, viii.

SOAP 2018