{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T19:48:39Z","timestamp":1769975319235,"version":"3.49.0"},"reference-count":49,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,2,12]],"date-time":"2014-02-12T00:00:00Z","timestamp":1392163200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Paroxysmal nocturnal hemoglobinuria (<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>) may present as cytopenia, hemolysis, or thrombosis at unusual sites including splanchnic vessels. Thrombosis of the portal veins and hepatic veins are associated with thrombophilic risk factors: deficiencies of protein C, protein S, and antithrombin, positivity for antiphospholipid antibodies, and factor V Leiden mutation. There is limited information regarding<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>presenting primarily as a thrombotic event. We prospectively screened 142 consecutive patients with intrabdominal thrombosis and 106 controls with fluorescently labeled inactive toxin aerolysin (<jats:styled-content style=\"fixed-case\">FLAER<\/jats:styled-content>)\u2010based flowcytometry to assess the frequency of<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>as a thrombophilic risk factor in patients with intra\u2010abdominal thrombosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Granulocytes of patients and controls were screened with<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>24 and<jats:styled-content style=\"fixed-case\">FLAER<\/jats:styled-content>and monocytes with<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>14 and<jats:styled-content style=\"fixed-case\">FLAER<\/jats:styled-content>. Dual negativity of &gt;1% events in both lineages was interpreted as a positive<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>clone. Screening for thrombophilia risk factors was carried out.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Two (1.4%) cases had large<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>clones.<jats:styled-content style=\"fixed-case\">RBC<\/jats:styled-content>also demonstrated the<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>defect. Thrombophilia risk factors were as follows: deficiency of protein S, protein C, and antithrombin in 13.4%, 4.9%, and 2.1%, respectively, and positivity for anti\u2010beta\u20102 glycoprotein 1, anticardiolipin antibodies, and lupus anticoagulant in 9.2%, 1.4%, and 0.7%, respectively. Factor V Leiden mutation was seen in 1.4% patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>was uncommon in patients with intra\u2010abdominal thrombosis in the ethnic Indian population. Despite low positivity, screening by flowcytometry for<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>is of value in this group of patients because it provides an opportunity to rapidly establish the diagnosis of this treatable disorder, which might otherwise be missed if the initial presentation is only thrombotic.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.12265","type":"journal-article","created":{"date-parts":[[2014,1,9]],"date-time":"2014-01-09T08:42:52Z","timestamp":1389256972000},"page":"435-443","source":"Crossref","is-referenced-by-count":16,"title":["Paroxysmal Nocturnal Hemoglobinuria is rare cause for thrombosis of the intra\u2010abdominal veins in the ethnic Indian population \u2013 results from<scp>FLAER<\/scp>\u2010based flowcytometry screening"],"prefix":"10.1111","volume":"92","author":[{"given":"Jasmina","family":"Ahluwalia","sequence":"first","affiliation":[{"name":"Department of Hematology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shano","family":"Naseem","sequence":"additional","affiliation":[{"name":"Department of Hematology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Man Updesh Singh","family":"Sachdeva","sequence":"additional","affiliation":[{"name":"Department of Hematology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Parveen","family":"Bose","sequence":"additional","affiliation":[{"name":"Department of Hematology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sunil Kumar","family":"Bose","sequence":"additional","affiliation":[{"name":"Department of Hematology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Narender","family":"Kumar","sequence":"additional","affiliation":[{"name":"Department of Hematology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Babu Ram","family":"Thapa","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neelam","family":"Varma","sequence":"additional","affiliation":[{"name":"Department of Hematology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yogesh Kumar","family":"Chawla","sequence":"additional","affiliation":[{"name":"Department of Hepatology Postgraduate Institute of Medical Education and Research Chandigarh India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,2,12]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v12.i8.1165"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90250-T"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2000.01802.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-04-1717"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199010253231707"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/cyto.b.20151"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V93.5.1749"},{"key":"e_1_2_7_9_1","first-page":"459","article-title":"Improved detection and characterization of paroxysmal nocturnal hemoglobinuria using fluorescent aerolysin","volume":"114","author":"Brodsky RA","year":"2000","journal-title":"Am J ClinPathol"},{"key":"e_1_2_7_10_1","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1002\/cyto.b.20525","article-title":"Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry","volume":"78","author":"Borowitz MJ","year":"2010","journal-title":"Cytometry B ClinCytom"},{"key":"e_1_2_7_11_1","first-page":"564","article-title":"Use of a FLAER\u2010based WBC assay in the primary screening of PNH clones","volume":"132","author":"Sutherland DR","year":"2009","journal-title":"Am J ClinPathol"},{"key":"e_1_2_7_12_1","volume-title":"Diseases of the Liver and Biliary System","author":"Sherlock S","year":"1996"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/369064a0"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0320(20000815)42:4<223::AID-CYTO2>3.0.CO;2-D"},{"key":"e_1_2_7_15_1","unstructured":"RosseWF.Paroxysmal Nocturnal HemoglobinuriaOrphanet Encyclopedia. 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