{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T03:40:09Z","timestamp":1778902809434,"version":"3.51.4"},"reference-count":44,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2012,5,29]],"date-time":"2012-05-29T00:00:00Z","timestamp":1338249600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2012,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The characteristics of Gaucher disease (<jats:styled-content style=\"fixed-case\">GD<\/jats:styled-content>) associated with persistent thrombocytopenia despite imiglucerase enzyme therapy in type 1 <jats:styled-content style=\"fixed-case\">GD<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">GD<\/jats:styled-content>1) were investigated by retrospective analysis of International Collaborative Gaucher Group (<jats:styled-content style=\"fixed-case\">ICGG<\/jats:styled-content>) Registry data. The study involved 1016 <jats:styled-content style=\"fixed-case\">GD<\/jats:styled-content>1 patients with an intact spleen for whom date of diagnosis, therapy initiation, and platelet counts were known, and who received continuous imiglucerase therapy for 4 to 5\u00a0years. These patients were stratified by last platelet count: \u2265120\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/l (<jats:italic>n\u00a0<\/jats:italic>=\u00a0772); \u2265100 to &lt;120\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/l (<jats:italic>n\u00a0<\/jats:italic>=\u00a094); \u226580 to &lt;100\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/l (<jats:italic>n\u00a0<\/jats:italic>=\u00a080); and &lt;80\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/l (<jats:italic>n\u00a0<\/jats:italic>=\u00a070; 20 with &lt;60\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/l) and characterized by initial and cumulative average imiglucerase dose, body mass index, platelet count, anaemia, hepatomegaly, splenomegaly, and skeletal assessments at baseline and after 4\u20135\u00a0years of therapy. Statistically significant associations were found between persistent thrombocytopenia and baseline platelet count (&lt;80\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/l), splenomegaly, and anaemia (all <jats:italic>P\u00a0<\/jats:italic>&lt;\u00a00\u00b70001). After 4\u20135\u00a0years, statistically significant associations were found with splenomegaly (<jats:italic>P\u00a0<\/jats:italic>&lt;\u00a00\u00b70001), anaemia (<jats:italic>P\u00a0<\/jats:italic>&lt;\u00a00\u00b70001), white blood cell count (<jats:italic>P\u00a0<\/jats:italic>=\u00a00\u00b7049), hepatomegaly (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7004) and bone pain (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7035). Exponential platelet decay in relation to splenomegaly suggests that platelets increase only when spleen volume decreases substantially.<\/jats:p>","DOI":"10.1111\/j.1365-2141.2012.09175.x","type":"journal-article","created":{"date-parts":[[2012,5,29]],"date-time":"2012-05-29T06:38:41Z","timestamp":1338273521000},"page":"528-538","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Characteristics of type I Gaucher disease associated with persistent thrombocytopenia after treatment with imiglucerase for 4\u20135\u00a0years"],"prefix":"10.1111","volume":"158","author":[{"given":"Carla E. 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Alexander","family":"Cole","sequence":"additional","affiliation":[{"name":"Genzyme, a Sanofi Company Cambridge MA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephan","family":"vom Dahl","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine St Franziskus Hospital Teaching Hospital University of Cologne Cologne Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick B.","family":"Deegan","sequence":"additional","affiliation":[{"name":"Department of Medicine Addenbrooke's Hospital Cambridge UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jack","family":"Goldblatt","sequence":"additional","affiliation":[{"name":"Genetic Services of Western Australia School of Paediatrics and Child Health University of Western Australia Perth WA Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barry","family":"Rosenbloom","sequence":"additional","affiliation":[{"name":"Tower Hematology 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