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Since routine coagulation parameters are often inconspicuous, diagnosis and treatment of the underlying coagulation disorders are challenging.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and methods<\/jats:title><jats:p>In our single\u2010center observational study, we analyzed 164 patients with MM for coagulation disorders and bleeding complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Prolonged closure times (CTs), measured by PFA\u2010100, were the most common, abnormal coagulation test, found in 66% of bleeding patients vs 5% in non\u2010bleeding, followed by qualitative defects of von Willebrand factor (VWF:CB\/VWF:Ag ratios), found in 34% vs 1% in the non\u2010bleeding group. Increased serum free light chains (SFLC) and SFLC ratios were significantly associated with prolonged CTs and acquired von Willebrand syndrome (AVWS). Prolonged CTs and AVWS were associated with disease progression, determined by dynamics of SFLC ratios (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.001), serum creatinine level (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.013), Beta\u20102 microglobulin (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.03), LDH (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.016), and bone marrow infiltration (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.001). Of note, response to myeloma therapy was frequently correlated with normalization of coagulation parameters.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Bleeding complications in MM are predominantly caused by defects in primary hemostasis and associated with disease progression. In a peri\u2010interventional workup, determination of CTs and VWF:CB\/VWF:Ag ratios are of significant importance to assess bleeding risk.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.13331","type":"journal-article","created":{"date-parts":[[2019,9,21]],"date-time":"2019-09-21T16:18:40Z","timestamp":1569082720000},"page":"26-35","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Disease progression and defects in primary hemostasis as major cause of bleeding in multiple myeloma"],"prefix":"10.1111","volume":"104","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2416-9399","authenticated-orcid":false,"given":"Clemens","family":"Hinterleitner","sequence":"first","affiliation":[{"name":"Department of Medical Oncology, Hematology, Immunology, Rheumatology and Pulmonology Eberhard\u2010Karls University Tuebingen 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