May-Hegglinova anomálie v těhotenství.2 kazuistiky

May-Hegglin Anomaly in Pregnancy. Two Case Report

Type of study:
Case report.Setting: Obstetrics and Gynecology Department, 2nd Medical Faculty Charles University and FacultyHospital Motol, Department of Hematology and blood transfusion, Obstetrics and GynecologyDepartment, 1st Medical Faculty Charles University and General Faculty Hospital, Prague.Methods: The authors present two cases of pregnant women with May-Hegglin anomaly. This rarehereditary trombocytopenia is characterized with the presence of megathrombocytes and typicalbasophilie inclusions (Dahli’s inclusions) in granulocyte cytoplasma in blood count. Clinicaly, thereare possible haemmorrhagic manifestations in this anomaly [13]. The course of pregnancy wasuncomplicated in both cases. The values of thrombocytes fluctuated between 22 - 34 x 109/l and 17 -27 x 109/l respectively. The response on the corticosteroid administration was minimal. The pregnancywas terminated with caesarean section in general anesthesia in both cases. The indication inthe first case was, after the neonatologist consultation with hematologist on duty, the anomaly itself.In the second case it was primarily decided to conduct the labor vaginaly. It was necessary to startthe induction of labor for the development preeclamptic signs. This prostaglandin induction wasunsuccessful and therefor was the labor also terminated with s.c. Both delivered neonates were ingood condition without clinical or lab signs of the disease. The neonate in the second case was ofborderline trophicity. The s.c. were secured with transfusions of trombocytes and carried outwithout any complications in both cases. The blood losses were 400 and 700 ml without necessity oferythrocyte transfusions. The postoperative course was also uncomplicated in both cases. Bothwomen with their children were released from hospital on the 6th postoperative day.Conclusion: There are about 20 published papers of authors describing pregnancy with this rareanomaly. The efforts of haemmorrhage are very rare, the course of pregnancy is usually notdisturbed [7, 8, 9, 11]. Some authors alert on possible higher risk intrauterine growth restriction [4].The risk of fetal hemorrhage is from all available, data also small and there is no need of prenatalinvasive investigation (cordocentesis) [11]. The mode of delivery depends only on the obstetrics andindications, vaginal labor is not associated with higher risk for the fetus [2, 3, 5, 6, 10]. The treatmentwith corticosteroids or immunoglobulins in cases when the clinical symptomatology appear is onlylittle effective, administration of thrombocytes transfusions are useful [8, 9]. When the anesthesiais necessary, general anesthesia is preferred because of higher risk of local haemmorrhage complicationsin cases of epidural or spinal analgesia [10, 14].

Key words:
May-Hegglin anomaly, trombocytopenia, pregnancy