Von Willebrand Factor and Von Willebrand Disease in Pregnancy – a Manageable Challenge

Repeated vaginal bleeding is a common complaint in outpatient obstetrical practice. Traditionally, it is treated with progesterone, without making an accurate etiological diagnosis. Hemorrhage during labor, delivery or postpartum period are considered obstetrical emergencies. Von Willebrand disease is one of the frequent causes for hereditary bleeding disorders; the diagnosis during pregnancy can be difficult due to physiological hematological changes. The aim of this study was to review the diagnostic criteria of von Willebrand disease and to recommended optimal management during pregnancy, delivery and postpartum period. The triad: family bleeding history, personal backgrounds of hemorrhage and easily appearing bruises after small trauma are suggestive for von Willebrand disease. However, the proper diagnosis is based on laboratory tests which consist of: von Willebrand-ristocetin cofactor activity, von Willebrand factor antigen, and factor VIII. In order to obtain optimal treatment outcomes, a multidisciplinary approach must include a hematologist as well. A prophylactic treatment with Haemate P® (CSL Behring, Marburg, Germany) is recommended in order to prevent any unwanted hemorrhagic events. Rezumat : Factorul von Willebrand si boala von Willebrand in sarcinao mare provocare In practica curenta, sangerarea vaginala repetata este un simptom comun. De obicei, se trateaza cu progestative si diagnosticul etiologic nu se stabileste intotdeauna. Hemoragia in timpul travaliului, nasterii si in perioada lauziei sunt considerate urgente obstetricale. Boala von Willebrand este una dintre cele mai frecvente cause ereditare de patologie hemoragica; in timpul sarcinii, datorita modificarilor hematologice fiziologice, diagnosticarea acesteia poate fi dificila. Scopul studiului a fost sa revedem criteriile de diagnostic ale bolii von Willebrand si sa recomandam o conduita optima in timpul supravegherii sarcinii, al nasterii si in perioada de lauzie. Triada: istoric familial de sangerare, istoric personal de hemoragii, aparitia echimozelor dupa traumatisme minore sugereaza boala von Willebrand. Diagnosticul este stabilit prin teste specifice de laborator precum: activitatea cofactorului von Willebrand la ristocetina, antigenul factorului von Willebrand , factorul VIII. O abordare multidisciplinara in care exista si medicul hematolog va asigura tratamentul optim. De fiecare data, pentru prevenirea hemoragiilor se va administra tratament cu Haemate P ® (CSL Behring, Marburg, Germany). Cuvinte cheie : boala von Willebrand , factorul von Willebrand, sarcina, Haemate P, hemoragie