[May-Heggelin anomaly and pregnancy. A case report, review of the literature].
The May-Hegglin anomaly is a rare autosomal dominant platelet disorder characterized by thrombocytopenia, giant platelets and existence of crescent-shaped inclusions within the cytoplasm of granulocytes, eosinophils and monocytes (Döhle body). We report a case of May-Hegglin anomaly associated with a pregnancy. The pregnancy and delivery were uneventful. The child is not a carrier of this hematologic anomaly. Nine cases of complicated pregnancies with this anomaly have been reported in the literature. The risks of maternal hemorrhagic accident during pregnancy and during delivery are weak due to the fact that platelets functions are preserved. The same applies to the fetus. Nevertheless, as in the case of maternal autoimmune thrombocytopenic purpura, most reports advice performing a fetal platelet count on fetal blood sampling before birth to decide upon the mode of delivery. The risk of the cordonal approach to perform fetal blood sampling must be balanced against the small fetal hemorrhagic risk and most authors propose to allow normal delivery whilst avoiding all traumatic instrumental extraction, especially the use of vacuum extractor.
Tordjeman
N
N
Service de Gynécologie-Obstétrique, Hôpital Jeanne-de-Flandre, CHRU, Lille.
Metzger
N
N
Valat
A S
AS
Dufour
P
P
Goudemand
J
J
Puech
F
F
D000328:Adult; D001792:Blood Platelets / Q000473:pathology*; D003594:Cytoplasmic Granules / Q000473:pathology*; D005260:Female; D005312:Fetal Blood; D005799:Genes, Dominant; D006098:Granulocytes / Q000473:pathology*; D006801:Humans; D009000:Monocytes / Q000473:pathology*; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000097:blood; D011256:Pregnancy Outcome; D013921:Thrombocytopenia / Q000097:blood / Q000235:genetics*