[The May-Hegglin anomaly in pregnancy. 2 case reports]

Case report.

Obstetrics and Gynecology Department, 2nd Medical Faculty Charles University and Faculty Hospital Motol, Department of Hematology and blood transfusion, Obstetrics and Gynecology Department, 1st Medical Faculty Charles University and General Faculty Hospital, Prague.

The authors present two cases of pregnant women with May-Hegglin anomaly. This rare hereditary trombocytopenia is characterized with the presence of megathrombocytes and typical basophilie inclusions (Dahli's inclusions) in granulocyte cytoplasma in blood count. Clinically, there are possible haemmorrhagic manifestations in this anomaly [13]. The course of pregnancy was uncomplicated in both cases. The values of thrombocytes fluctuated between 22-34 x 10(9)/l and 17-27 x 10(9)/l respectively. The response on the corticosteroid administration was minimal. The pregnancy was terminated with caesarean section in general anesthesia in both cases. The indication in the 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 vaginally. It was necessary to start the induction of labor for the development preeclamptic signs. This prostaglandin induction was unsuccessful and therefor was the labor also terminated with s.c. Both delivered neonates were in good condition without clinical or lab signs of the disease. The neonate in the second case was of borderline trophicity. The s.c. were secured with transfusions of thrombocytes and carried out without any complications in both cases. The blood losses were 400 and 700 ml without necessity of erythrocyte transfusions. The postoperative course was also uncomplicated in both cases. Both women with their children were released from hospital on the 6th postoperative day.

There are about 20 published papers of authors describing pregnancy with this rare anomaly. The efforts of haemmorrhage are very rare, the course of pregnancy is usually not disturbed [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 prenatal invasive investigation (cordocentesis) [11]. The mode of delivery depends only on the obstetrics and indications, vaginal labor is not associated with higher risk for the fetus [2, 3, 5, 6, 10]. The treatment with corticosteroids or immunoglobulins in cases when the clinical symptomatology appear is only little effective, administration of thrombocytes transfusions are useful [8, 9]. When the anesthesia is necessary, general anesthesia is preferred because of higher risk of local haemmorrhage complications in cases of epidural or spinal analgesia [10, 14].