Prevención de la hemorragia posparto en una gestante afectada de síndrome de Hermansky-Pudlack

Hermansky-Pudlak syndrome is a multisystemic disease with autosomal recessive inheritance, mainly characterized by oculo-cutaneous albinism and impaired platelet aggregation. We describe the follow-up and end of pregnancy in a 30-year-old woman with this syndrome, as well as the measures carried out during labor to avoid bleeding complications due to platelet dysfunction. The pregnancy ended at 38.2 weeks through vaginal delivery, without epidural anesthesia and good maternal and fetal outcome.