{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T18:29:41Z","timestamp":1793644181680,"version":"4.5.1"},"reference-count":53,"publisher":"American Society of Hematology","issue":"25","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,6,19]]},"abstract":"<jats:sec>\n                  <jats:title>Abstract<\/jats:title>\n                  <jats:p>Type 1 plasminogen deficiency (PLGD), an ultrarare disorder caused by PLG pathogenic variants, results in decreased levels of immunoreactive and functional plasminogen. PLGD can cause fibrin-rich pseudomembranes on mucosa that impair tissue\/organ function, affect quality of life, and are potentially life threatening. Lesion regression\/resolution is facilitated by IV administration of human plasma-derived Glu-plasminogen (IV PLG concentrate), the first and only US Food and Drug Administration\u2013approved specific treatment, licensed in 2021. The diagnosis of PLGD is frequently delayed because of its rarity (1.6 per million) and the variability of the initial medical specialty contact determined by the affected systems. Symptoms are often attributed to more common conditions, such as conjunctivitis, recurrent otitis media, reactive airway disease, etc. This article presents clinical vignettes highlighting strategies for PLGD diagnosis and treatment. Initial evaluation includes a detailed history, laboratory assays, and, at times, radiologic or other procedures. Genetic testing can confirm the diagnosis. Consistent, knowledgeable management is required to promptly identify and treat lesions, even in initially asymptomatic individuals. Personalized treatment may include continuous prophylaxis or intermittent treatment with IV PLG concentrate, dependent on disease severity and clinical course. Specialized facilities such as hemophilia treatment centers offering multidisciplinary care represent medical homes for this ultrarare disorder.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1182\/blood.2024026973","type":"journal-article","created":{"date-parts":[[2025,3,16]],"date-time":"2025-03-16T20:54:56Z","timestamp":1742158496000},"page":"2954-2965","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":8,"title":["How I treat type 1 plasminogen deficiency"],"prefix":"10.1182","volume":"145","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2821-7159","authenticated-orcid":false,"given":"Amy D.","family":"Shapiro","sequence":"first","affiliation":[{"name":"Indiana Hemophilia and Thrombosis Center, Indianapolis, IN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Nakar","sequence":"additional","affiliation":[{"name":"Indiana Hemophilia and Thrombosis Center, Indianapolis, IN"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"issue":"4","key":"2025061911212565200_bib1","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1016\/S0039-6257(03)00056-0","article-title":"Ligneous conjunctivitis","volume":"48","author":"Schuster","year":"2003","journal-title":"Surv Ophthalmol"},{"issue":"10","key":"2025061911212565200_bib2","doi-asserted-by":"crossref","first-page":"1368","DOI":"10.1111\/j.1468-3083.2006.01722.x","article-title":"Juvenile colloid milium and ligneous conjunctivitis are caused by severe hypoplasminogenemia \u2013 no evidence for causal relationship to non-Hodgkin's lymphoma","volume":"20","author":"Schuster","year":"2006","journal-title":"J Eur Acad Dermatol Venereol"},{"issue":"9","key":"2025061911212565200_bib3","doi-asserted-by":"crossref","first-page":"3021","DOI":"10.1182\/blood-2006-04-017350","article-title":"Molecular and clinical spectrum of type I plasminogen deficiency: a series of 50 patients","volume":"108","author":"Tefs","year":"2006","journal-title":"Blood"},{"issue":"3","key":"2025061911212565200_bib4","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1182\/blood.V90.3.958","article-title":"Homozygous mutations in the plasminogen gene of two unrelated girls with ligneous conjunctivitis","volume":"90","author":"Schuster","year":"1997","journal-title":"Blood"},{"issue":"3","key":"2025061911212565200_bib5","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1055\/s-2007-996099","article-title":"Homozygous type I plasminogen deficiency","volume":"23","author":"Mingers","year":"1997","journal-title":"Semin Thromb Hemost"},{"issue":"3","key":"2025061911212565200_bib6","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1042\/bj3390705","article-title":"Extrahepatic synthesis of plasminogen in the human cornea is up-regulated by interleukins-1\u03b1 and -1\u03b2","volume":"339","author":"Twining","year":"1999","journal-title":"Biochem J"},{"issue":"3","key":"2025061911212565200_bib7","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1055\/s-0037-1613030","article-title":"Plasminogen has a broad extrahepatic distribution","volume":"87","author":"Zhang","year":"2002","journal-title":"Thromb Haemost"},{"issue":"3","key":"2025061911212565200_bib8","doi-asserted-by":"crossref","first-page":"445","DOI":"10.3390\/cells12030445","article-title":"The role of the plasminogen\/plasmin system in inflammation of the oral cavity","volume":"12","author":"Yatsenko","year":"2023","journal-title":"Cells"},{"issue":"4447","key":"2025061911212565200_bib9","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1126\/science.6990488","article-title":"Synthesis of human plasminogen by the liver","volume":"208","author":"Raum","year":"1980","journal-title":"Science"},{"issue":"suppl 1","key":"2025061911212565200_bib10","first-page":"7","article-title":"Plasminogen: a brief introduction into its biochemistry and function","volume":"18","author":"Miyashita","year":"1988","journal-title":"Haemostasis"},{"issue":"11","key":"2025061911212565200_bib11","doi-asserted-by":"crossref","first-page":"4044","DOI":"10.1016\/S0021-9258(19)41383-5","article-title":"Physical and chemical properties of the NH2-terminal glutamic acid and lysine forms of human plasminogen and their derived plasmins with an NH2-terminal lysine heavy (A) chain","volume":"250","author":"Robbins","year":"1975","journal-title":"J Biol Chem"},{"issue":"5","key":"2025061911212565200_bib12","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1172\/JCI113790","article-title":"Endothelial cell-mediated conversion of Glu-plasminogen to Lys-plasminogen. 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