{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T19:18:07Z","timestamp":1793215087944,"version":"4.5.0"},"reference-count":37,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,3,9]],"date-time":"2021-03-09T00:00:00Z","timestamp":1615248000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["obgyn.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Intl J Gynecology &amp; Obste"],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hermansky\u2010Pudlak syndrome (HPS) is a rare autosomal\u2010recessive disorder with clinical manifestations of bleeding diathesis, multi\u2010organ disease and variable oculocutaneous albinism (OCA). In women, it can cause life\u2010threatening obstetric and gynecological (OB\/GYN) bleeding.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To summarize OB\/GYN presentations, outcomes, and management strategies in women with HPS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Search strategy<\/jats:title>\n                    <jats:p>Main databases (MEDLINE, EMBASE, Cochrane, PubMed, Web of Science Core Collection and Google Scholar) were searched from inception until June 30, 2020.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Selection criteria<\/jats:title>\n                    <jats:p>Case reports\/series of women with confirmed HPS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Data collection and analysis<\/jats:title>\n                    <jats:p>A systematic review using PRISMA guidelines. Methodological quality assessment performed using adapted Newcastle Ottawa scale.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Main results<\/jats:title>\n                    <jats:p>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\u2010partum 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ijgo.13632","type":"journal-article","created":{"date-parts":[[2021,2,1]],"date-time":"2021-02-01T01:57:40Z","timestamp":1612144660000},"page":"412-426","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The presentation and outcomes of Hermansky\u2010Pudlak syndrome in obstetrics and gynecological settings: A systematic review"],"prefix":"10.1002","volume":"154","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0334-1121","authenticated-orcid":false,"given":"Deborah","family":"Obeng\u2010Tuudah","sequence":"first","affiliation":[{"name":"Katharine Dormandy Haemophilia and Thrombosis Centre Royal Free Hospital NHS Trust London UK"},{"name":"Department of Obstetrics and Gynaecology Royal Free Hospital NHS Trust London UK"},{"name":"EGA Institute for Women\u2019s Health University College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brwa A.","family":"Hussein","sequence":"additional","affiliation":[{"name":"Katharine Dormandy Haemophilia and Thrombosis Centre Royal Free Hospital NHS Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amir","family":"Hakim","sequence":"additional","affiliation":[{"name":"Barts and the London School of Medicine and Dentistry Queen Mary University of London London UK"},{"name":"National Heart and Lung Institute Imperial College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keith","family":"Gomez","sequence":"additional","affiliation":[{"name":"Katharine Dormandy Haemophilia and Thrombosis Centre Royal Free Hospital NHS Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2684-1006","authenticated-orcid":false,"given":"Rezan","family":"Abdul Kadir","sequence":"additional","affiliation":[{"name":"Katharine Dormandy Haemophilia and Thrombosis Centre Royal Free Hospital NHS Trust London UK"},{"name":"Department of Obstetrics and Gynaecology Royal Free Hospital NHS Trust London UK"},{"name":"EGA Institute for Women\u2019s Health University College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,3,9]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V14.2.162.162"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1616221"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199804303381803"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaapos.2013.05.012"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.179.4.1790887"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2014.147"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.4103\/2156-7514.143437"},{"key":"e_1_2_8_9_1","first-page":"333","article-title":"Albinism and Hermansky\u2010Pudlak syndrome in Puerto Rico","volume":"82","author":"Witkop CJ","year":"1990","journal-title":"Bol Assoc Med PR"},{"key":"e_1_2_8_10_1","unstructured":"HuizingM MalicdanMC GochuicoBR GahlWA.Hermansky Pudlak syndrome. GeneReviews. Initial posting: July 24 2000. Updated: October 26 2017.https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1287\/. Accessed November 7 2020."},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12064"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830260403"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jclinepi.2009.06.005"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pog.2011.12.008"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjebm-2017-110853"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/bja\/aeh256"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0301-2115(02)00063-5"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.3109\/01443615.2011.638090"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2001-14528"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0029-1220777"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.7205\/MILMED.173.10.1048"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13458"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9378(85)90475-2"},{"issue":"5","key":"e_1_2_8_24_1","first-page":"267","article-title":"Hermansky\u2010Pudlak syndrome in a pregnant patient: a case report","volume":"58","author":"Harris\u2010Glocker M","year":"2013","journal-title":"J Reprod Med"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00404-013-3110-7"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1258\/om.2008.080003"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr\u20102016\u2010217719"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1177\/1753495X16662580"},{"issue":"1","key":"e_1_2_8_29_1","first-page":"S61","article-title":"Therapy refractory menorrhagia as first manifestation of Hermansky\u2010Pudlak syndrome","volume":"31","author":"Lohse J","year":"2011","journal-title":"Hamostaseologie"},{"issue":"9","key":"e_1_2_8_30_1","first-page":"2023","article-title":"Case report: Hermansky Pudlak syndrome (presenting as late onset heavy menstrual bleeding)","volume":"7","author":"Ray A","year":"2013","journal-title":"J Clin Diagn Res"},{"key":"e_1_2_8_31_1","unstructured":"Royal College of Obstetricians and Gynaecologists.Prevention and management of postpartum haemorrhage. Green\u2010top guideline no 52.2016.https:\/\/www.rcog.org.uk\/en\/guidelines\u2010research\u2010services\/guidelines\/gtg52\/. Accessed March 30 2020."},{"key":"e_1_2_8_32_1","first-page":"2","article-title":"The age of menarche","volume":"4","author":"Rees M","year":"1995","journal-title":"ORGYN"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04286.x"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1136\/ebmental\u20102019\u2010300129"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02573.x"},{"key":"e_1_2_8_36_1","first-page":"278","article-title":"Inherited platelet disorders: thrombocytopenias and thrombocytopathies","volume":"7","author":"D\u2019Andrea G","year":"2009","journal-title":"Blood Transf"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006254-199807000-00025"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/1471-0528.14592"}],"container-title":["International Journal of Gynecology &amp; Obstetrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ijgo.13632","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ijgo.13632","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ijgo.13632","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T21:06:11Z","timestamp":1764968771000},"score":1,"resource":{"primary":{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/10.1002\/ijgo.13632"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,9]]},"references-count":37,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2021,9]]}},"alternative-id":["10.1002\/ijgo.13632"],"URL":"https:\/\/doi.org\/10.1002\/ijgo.13632","archive":["Portico"],"relation":{},"ISSN":["0020-7292","1879-3479"],"issn-type":[{"value":"0020-7292","type":"print"},{"value":"1879-3479","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,9]]},"assertion":[{"value":"2020-08-05","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-01-28","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-03-09","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}