{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T23:34:08Z","timestamp":1740180848025,"version":"3.37.3"},"reference-count":31,"publisher":"Consilium Medicum","issue":"9","license":[{"start":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T00:00:00Z","timestamp":1698969600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Terapevticheskii arkhiv"],"abstract":"<jats:p>Aim. To analyze the frequency and nature of hemorrhagic and thrombotic complications in patients with systemic AL-amyloidosis and compare with laboratory changes in the hemostasis system.\r\nMaterials and methods. The prospective study included 40 patients with newly diagnosed AL-amyloidosis. To detect amyloid, all patients underwent bone marrow trephine biopsy and duodenal biopsy, and 28 (70%) patients underwent biopsy of the affected organ. Before the start of therapy, all patients were determined the platelet count, activated partial thromboplastin time, thrombin time, fibrinogen concentration, time of XIIa-dependent fibrinolysis, antithrombin III, D-dimer, activity of blood coagulation factors VIII, X and vWF. The statistical part of the study was carried out using the IBM SPSS Statistics 2017 system software (SPSS, Chicago, IL, USA).\r\nResults. In 20 (50%) patients, hemorrhages on the skin and mucous membranes were diagnosed as vascular purpura. Before the start of therapy, 7 (17.5%) patients had thrombosis, including leg vein thrombosis (5 patients), ischemic stroke (2 patients). There was a direct correlation between thrombotic complications and cutaneous hemorrhagic syndrome (p=0.007). In 15 (75%) cases, cutaneous hemorrhagic syndrome was accompanied by hypercoagulable shifts in the hemostasis system. Of the 20 patients with cutaneous hemorrhagic syndrome, 19 (95%) patients had kidney damage, including 15 patients with nephrotic syndrome. Hematoma type of bleeding, as well as heavy bleeding was not observed, including after a biopsy of the internal organs. According to the totality of hemostasis indicators, hypercoagulation syndrome was more often observed (in 23; 56% of patients). Hypocoagulation was diagnosed only in 2 (5%) patients with liver damage, 16 (39%) patients had normocoagulation.\r\nConclusion. Cutaneous hemorrhagic syndrome is the most common clinical manifestation of disorders in the hemostasis system in patients with AL-amyloidosis. The relationship of hemorrhages on the skin with nephrotic syndrome has been established, which may indicate a single pathogenetic mechanism. Cutaneous hemorrhagic syndrome is associated with hypercoagulable shifts in hemostasis and a high risk of thrombotic complications.<\/jats:p>","DOI":"10.26442\/00403660.2023.09.20237","type":"journal-article","created":{"date-parts":[[2023,11,4]],"date-time":"2023-11-04T15:47:14Z","timestamp":1699112834000},"page":"746-750","source":"Crossref","is-referenced-by-count":0,"title":["Hemostasis disorders in patients with systemic AL-amyloidosis"],"prefix":"10.26442","volume":"95","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5440-4340","authenticated-orcid":false,"given":"Irina G.","family":"Rekhtina","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1008-5007","authenticated-orcid":false,"given":"Victoria A.","family":"Khyshova","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7074-0926","authenticated-orcid":false,"given":"Nadezhda I.","family":"Zozulya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9877-0796","authenticated-orcid":false,"given":"Valentina N.","family":"Dvirnyk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4966-8146","authenticated-orcid":false,"given":"Larisa P.","family":"Mendeleyeva","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"18344","published-online":{"date-parts":[[2023,11,3]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2018.08.006"},{"key":"ref2","doi-asserted-by":"crossref","unstructured":"Yood RA, Skinner M, Rubinow A, Talarico L. CAS. Bleeding manifestations in 100 patients with amyloidosis. J Am Med Assoc. 1983;10(249):1922-324.","DOI":"10.1001\/jama.1983.03330340064034"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1056\/nejmicm061510"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13155"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.tripleo.2006.01.003"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2020.06.061"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.hemonc.2018.05.002"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.4081\/hr.2018.7235"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1097\/00001721-200209000-00011"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-004-0970-8"},{"key":"ref11","unstructured":"Gamba G, Montani N, Anesi E, et al. Clotting alterations in primary systemic amyloidosis. 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