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

Anesthetic Management of a Parturient with Lyonized Hemophilia A

Abstract Number: T1D-3
Abstract Type: Case Report/Case Series

Kristen Fardelmann M.D.1 ; Kathleen Smith M.D.2

Intro: Hemophilia A is a X-linked congenital bleeding disorder leading to deficiency of Factor VIII (FVIII). Female carriers are expected to have 50% normal factor activity, which is usually sufficient to prevent clinical bleeding. In a small subset of women (<0.001%), there is X-inactivation of the normal allele (lyonization), resulting in symptomatic bleeding. These patients require a comprehensive, multidisciplinary approach to prevent morbidity and mortality in both mother and fetus.

Case: The patient is a 25 year-old G2P1 admitted for induction of labor at 39 weeks in the setting of lyonization in a hemophilia A carrier. The patient reported a history of hemarthrosis, menorrhagia, and bleeding with procedures. Genetic testing was consistent with a single-base mutation that encodes a missense mutation in the translated FVIII protein. The patient’s laboratory values revealed a baseline FVIII activity of 20%, consistent with mild hemophilia, which increased to 71% at term. Her vWF activity, coagulation studies, platelet function, and factor VIII inhibitor testing were normal. The patient received FVIII concentrate dosed at about 15 units/kg every 12 hours peripartum. An epidural was placed for labor analgesia after the initial FVIII dose. A live born female was vaginally delivered with APGARS 9 and 9 and minimal blood loss. Prevention of secondary hemorrhage was provided by 15 units/kg every other day for 1 week. The patient declined postpartum antifibrinolytic therapy. Her postpartum course was uneventful.

Discussion: In pregnancy, FVIII activity levels peak at term. The severity of bleeding inversely correlates with the factor level. There are no recommendations for neuraxial placement, although a FVIII activity level of at least 50% has been considered adequate. Each unit of FVIII concentrate infused per kg of body weight will raise the plasma FVIII level about 2 IU/dL (2%) in the absence of an inhibitor with a half-life of 8-12 hours. Although a previous trial did not elicit a response, DDAVP given at 0.3 mcg/kg may raise FVIII in patients with mild and moderate disease. DDAVP has been previously used safely in the peripartum period. Tranexamic acid and epsilon aminocaproic acid help to promote clot stability and were recommended to our patient for the first week postpartum. Avoidance of NSAIDs and aspirin, operative vaginal delivery, intramuscular injection of medications, use of fetal scalp electrodes, and fetal venous sampling is paramount. Parturients should be delivered at a facility with a comprehensive hematology service with availability of factor concentrates and access to adequate laboratory support. Delivery should be scheduled when resources are optimally available. A history of excessive bleeding should be elicited from all hemophilia A carriers and trigger suspicion of low FVIII activity levels.

1. Srivastava et al. Haemophilia. 2013.

2. Sharma et al. Am J Perinatol Rep. 2013.

3. Dhar et al. Br J Anaesth. 2003.

SOAP 2018