HEREDITARY MACROTHROMBOCYTOPENIA AND PREGNANCY

Introduction. Hereditary macrothrombocytopenias (HM) are a group of in frequent disorders characterized by hereditary giant plate lets. Littl e has been published about the course of these diseases during pregnan cy and delivery. Subjects and methods. Forty consecutive thrombocytope nic pregnant women were studied. Platelet count, mean platelet volume and blood smear examination were performed. Platelet antibodies were s tudied by immunofluorescence. Familial study, bleeding time, ultrastru ctural platelet examination, a von Willebrand disease screening and ag gregation tests were carried out when HM was suspected. Results. Four cases of HM were diagnosed. Giant platelets were observed in all cases , with the typical ultrastructural pattern. Dohle-like cytoplasmic inc lusions in granulocytes were observed in one case. Platelet antibodies were detected in only one case. No prophylactic measures to prevent h aemorrhage were adopted, and all patients underwent vaginal deliveries . Haemorrhagic events were absent in both mothers and children. Conclu sions. The prevalence of HM in pregnant trombocytopenic women is highe r than assumed. Prophylactic treatment should be avoided in the absenc e of a history of haemorrhagic complications and obstetrical risk fact ors.