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The presentation and outcomes of Hermansky‐Pudlak syndrome in obstetrics and gynecological settings: A systematic review
Author(s) -
ObengTuudah Deborah,
Hussein Brwa A.,
Hakim Amir,
Gomez Keith,
Abdul Kadir Rezan
Publication year - 2021
Publication title -
international journal of gynecology and obstetrics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.895
H-Index - 97
eISSN - 1879-3479
pISSN - 0020-7292
DOI - 10.1002/ijgo.13632
Subject(s) - medicine , desmopressin , hermansky–pudlak syndrome , obstetrics , cochrane library , pregnancy , gynecology , medline , pediatrics , meta analysis , pulmonary fibrosis , biology , genetics , fibrosis , political science , law
Abstract Background Hermansky‐Pudlak syndrome (HPS) is a rare autosomal‐recessive disorder with clinical manifestations of bleeding diathesis, multi‐organ disease and variable oculocutaneous albinism (OCA). In women, it can cause life‐threatening obstetric and gynecological (OB/GYN) bleeding. Objective To summarize OB/GYN presentations, outcomes, and management strategies in women with HPS. Search strategy Main databases (MEDLINE, EMBASE, Cochrane, PubMed, Web of Science Core Collection and Google Scholar) were searched from inception until June 30, 2020. Selection criteria Case reports/series of women with confirmed HPS. Data collection and analysis A systematic review using PRISMA guidelines. Methodological quality assessment performed using adapted Newcastle Ottawa scale. Main results A total 29 pregnancies in 15 women and 2 gynecological patients were identified. Heavy menstrual bleeding (HMB), the most common bleeding symptom, was reported in 8/15 (53%) of women. HMB and post‐partum hemorrhage (PPH) led to diagnosis of HPS in 5/17 (29%) women. Primary PPH was reported in 12/27 (44%) of viable pregnancies; half were major PPH. In 17 pregnancies with known HPS diagnosis, 9 had hemostatic cover with desmopressin and 8 with platelet transfusion. Major PPH occurred in 3/9 (33%) pregnancies covered with desmopressin compared with none in the platelet group. Conclusion Diagnosis of HPS should be considered in women with OCA presenting with HMB or PPH. Hemostatic management options include desmopressin and platelet transfusion. Management should be multidisciplinary with close collaboration between OB/GYN and hematology teams.