TITLE:
Case of Refractory Thrombocytopenia in Pregnancy Associated with May-Heglin Anomaly after Repeated Platelet Transfusions
AUTHORS:
Aalaa A. Wahab Marzooq, Aseel Ahmed Husain, Aayat Jaaffar Naseeb
KEYWORDS:
Platelets, Pregnancy, Macrothrombocytopenia, MHA
JOURNAL NAME:
Open Journal of Obstetrics and Gynecology,
Vol.14 No.7,
July
19,
2024
ABSTRACT: May-Heglin Anomaly is an autosomal dominant disorder characterized by macrothrombocytopenia with a platelet function that is usually preserved. Platelets play an essential role in hemostasis. During pregnancy, a woman is susceptible to complications, including postpartum hemorrhage. Monitoring patients’ hemostatic functions and observing the patient’s clinical picture to maintain patient safety is paramount, while avoiding unnecessary therapeutic measures. This case report presents a rare instance of May-Heglin Anomaly (MHA) in a 35-year-old pregnant patient, with refractory thrombocytopenia despite receiving multiple platelet transfusions. Initially referred to as gravida 5 para 4 with severe thrombocytopenia at 28 weeks gestation, throughout her pregnancy, she was closely monitored and received over 40 units of platelets, which failed to increase her platelet count significantly. She delivered a healthy baby via vaginal delivery at 38 weeks, with her platelet count still critically low. This report highlights the challenges of managing MHA in pregnancy, the inefficacy of standard thrombocytopenia treatments such as platelet transfusion in MHA patients, and the importance of tailored management strategies to ensure maternal and fetal safety.