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In the present retrospective study, we aimed to investigate coagulation disorders and their outcome implications in patients with secondary HLH. We evaluated clinical characteristics and the relationship between coagulation indices and prognosis in HLH patients (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u2009141). The information, including clinical symptoms, laboratory indicators, and coagulation indices, was evaluated. Coagulation disorders and bleeding events occurred in 95 (67.4%) and 60 (42.6%) patients, respectively. A coagulation index analysis primarily showed elevated levels of D-Dimer, the international standardized ratio (INR), prothrombin time (PT), activated partial thromboplastin time (APTT), and thrombin time (TT), while the prothrombin activity, fibrinogen levels, and platelet levels were significantly decreased. Dominant disseminated intravascular coagulation (DIC) occurred in 76 patients (53.9%). Patients with lymphoma-associated hemophagocytic syndrome (LAHS) frequently exhibited apparent coagulation disorders. Multivariate analysis revealed that age\u2009\u2265\u200929.5\u00a0years, bleeding events, APTT\u2009\u2265\u200947.3\u00a0s, fibrinogen\u2009\u2264\u20091.68\u00a0g\/L, and absolute neutrophil counts (ANC) of\u2009\u2264\u20091.21\u2009\u00d7\u200910\n                    <jats:sup>9<\/jats:sup>\n                    \/L were independent prognostic factors. We thereby devised a prognostic scoring system and stratified patients into low-risk (0\u20132 points), intermediate-risk (3\u20134 points), and high-risk (5\u20137 points) groups, and the 1-year overall survival rates in the above-mentioned groups were 66.40%, 40.00%, and 2.30%, respectively (\n                    <jats:italic>P<\/jats:italic>\n                    \u2009&lt;\u20090.0001). In conclusion, coagulation dysfunctions and bleeding tendencies were common characteristics in HLH patients. We constructed a novel prognostic score model based on APTT, fibrinogen level, ANC, age, and bleeding events, which had superior prognostic value compared with these markers alone.\n                  <\/jats:p>","DOI":"10.1007\/s00277-023-05398-w","type":"journal-article","created":{"date-parts":[[2023,8,10]],"date-time":"2023-08-10T02:02:54Z","timestamp":1691632974000},"page":"3251-3259","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["A novel prognosis-prediction model based on coagulation indicators in secondary hemophagocytic lymphohistiocytosis"],"prefix":"10.1007","volume":"102","author":[{"given":"Shixuan","family":"Wang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kebing","family":"Lv","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yulan","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoye","family":"Cheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhiwei","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huimin","family":"Shen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fei","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,8,10]]},"reference":[{"key":"5398_CR1","doi-asserted-by":"publisher","first-page":"1253","DOI":"10.1016\/j.jpeds.2013.06.053","volume":"163","author":"S Chandrakasan","year":"2013","unstructured":"Chandrakasan S, Filipovich AH (2013) Hemophagocytic lymphohistiocytosis: Advances in pathophysiology, diagnosis, and treatment. 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Author Kebing Lv declares that she has no conflict of interest. Author Yulan Zhou declares that she has no conflict of interest. Author Xiaoye Cheng declares that she has no conflict of interest. Author Zhiwei Chen declares that he has no conflict of interest. Author Huimin Shen declares that she has no conflict of interest. Author Fei Li declares that she has no conflict of interest.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}