{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T21:13:15Z","timestamp":1777756395458,"version":"3.51.4"},"reference-count":0,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2009,2,25]],"date-time":"2009-02-25T00:00:00Z","timestamp":1235520000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2009,2,25]],"date-time":"2009-02-25T00:00:00Z","timestamp":1235520000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Haematol"],"abstract":"<jats:p>26 patients, 13 male and 13 female, with paroxysmal nocturnal haemoglobinuria (PNH) are described. The diagnosis was based on the finding of a positive Ham\u2019s test. PNH developed in 4 patients with aplastic anaemia, and 3 patients with established PNH developed marrow hypoplasia during the course of the disease. In 2 cases autoimmune haemolysis was also present; this association has not been described previously. The majority of patients presented with anaemia and dark urine, or with evidence of thrombosis. A high index of suspicion was needed to avoid missing the diagnosis. Haemolytic crises were usually precipitated by infection, and renal failure requiring dialysis sometimes resulted; a positive direct antiglobulin test was often found at times of increased haemolysis. Thromboses were the most frequent complication, and when intra-abdominal vessels were affected, pain was particularly troublesome. The disease had a widely variable course; 4 patients made a complete recovery and 10 died, 8 from thrombotic complications and 2 from infections associated with marrow hypoplasia. Survival ranged from 1 year to 30 years and the median survival in those who died was 3.5 years.<\/jats:p>","DOI":"10.1159\/000206591","type":"journal-article","created":{"date-parts":[[2009,2,27]],"date-time":"2009-02-27T04:37:06Z","timestamp":1235709426000},"page":"217-226","source":"Crossref","is-referenced-by-count":17,"title":["Paroxysmal Nocturnal Haemoglobinuria"],"prefix":"10.1159","author":[{"given":"K.","family":"Forman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.J.","family":"Sokol","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Hewitt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara K.","family":"Stamps","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2009,2,25]]},"container-title":["Acta Haematologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/206591","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000206591","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T12:26:32Z","timestamp":1777465592000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000206591"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,2,25]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1159\/000206591","archive":["Portico"],"relation":{},"ISSN":["0001-5792","1421-9662"],"issn-type":[{"value":"0001-5792","type":"print"},{"value":"1421-9662","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,2,25]]}}}