Peripartum management of a patient with a May-Hegglin Anomaly: A case report

May-Hegglin Anomaly is a rare, autosomal-dominant cause of thrombocytopenia in the family of myosin heavy chain-related disorders. A trademark of this anomaly is a low platelet count without platelet destruction, resulting in generally stable platelet counts throughout life. There is a spectrum of disease severity, ranging from mild bleeding to severe post-operative hemorrhage. To date, there are no guidelines on transfusion management or recommended platelet counts for neuraxial anesthesia in patients with myosin heavy-chain related disorders. We present the case of a 31-year-old primigravida with May-Hegglin Anomaly, gestational hypertension, and gestational diabetes mellitus requiring insulin who presented for scheduled induction of labor at 37-weeks gestation. On admission, she met criteria for preeclampsia with severe features and had a platelet count of 46,000/mm 3 . Following platelet transfusion, her platelets reached 77,000/mm 3 and a dural puncture epidural was placed. The patient was indicated for an urgent cesarean delivery after reexamination showed breech presentation. In the operating room, she was converted to general anesthesia due to concern for high anesthetic level. The patient experienced multiple platelet count fluctuations throughout her peripartum course. She required a total of 8 platelet transfusions over 34 h to maintain a neuraxial anesthesia platelet goal of ≥70,000/mm 3 . This case highlights that the characteristically stable platelet counts in May-Hegglin Anomaly become less reliable in the presence of other platelet-modifying diseases such as preeclampsia. Clinicians should counsel parturients with May-Hegglin Anomaly on the risks and benefits of multiple platelet transfusions in the event that the patient's peripartum course involves post-transfusion platelet fluctuations.