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

The presentation and outcomes of Hermansky‐Pudlak syndrome in obstetrics and gynecological settings: A systematic review

“…Hemostatic cover immediately after delivery and the puerperium were only reported in 6/27 (22%) cases. TXA was continued as prophylaxis in three cases 13,17,27 . In the immediate postpartum period, one pregnancy received an additional dose of desmopressin and two pregnancies received further units of platelets 24,26 …”

“…One article was written in Spanish, so we obtained its translation into English. 13 The other 15 articles were published in English.…”

“…The data extracted from the cases are represented in Tables 1 and 2. 13,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29] In all the studies, diagnosis of HPS was confirmed by a hematologist; of which nine (56%) studies fully described the diagnostic criteria met by subject(s). Further subgroup analysis revealed a high percentage of studies (n = 15; 94%) adequately described and reported study outcome(s) and ensured adequate subject follow-up.…”

“…TXA was continued as prophylaxis in three cases. 13,17,27 In the immediate postpartum period, one pregnancy received an additional dose of desmopressin and two pregnancies received further units of platelets. 24,26…”

“…22 Pregnancies managed with hemostatic platelet transfusion had lower EBL (8 cases, highest EBL 800 ml) compared to those managed with desmopressin (9 cases, highest EBL 1600 ml). 13,[16][17][18][19][20][21][22][23][24][25][26][27] Major PPH occurred in 3/9 (33%) cases in the desmopressin group compared to none when platelets were used. Platelets and packed red cells transfusion were required to treat PPH in 4/9 (44%) and 2/9 (22%) cases in the desmopressin group respectively.…”