{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T11:47:19Z","timestamp":1782906439087,"version":"3.54.5"},"reference-count":17,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Reports"],"abstract":"<jats:p>Background and Clinical Significance: Hyperviscosity syndrome (HVS) is a rare complication of primary Sj\u00f6gren\u2019s syndrome (pSS). While therapeutic plasma exchange (TPE) is the standard treatment to clear pathogenic immunoglobulins, its execution can trigger severe, atypical systemic risks. Case Presentation: A 60-year-old woman with pSS and extreme polyclonal hypergammaglobulinemia (total protein 100 g\/L, IgM 41 g\/L) presented with an acute hyperviscosity crisis, causing retinopathy, neurological deficits, and skin ischemia. Emergency TPE with 5% albumin replacement successfully reduced IgM by ~90% (to 6.39 g\/L), resolving HVS symptoms. However, 20 min post-procedure, the patient suffered sudden hemodynamic collapse (BP 50\/30 mmHg) and developed multiple massive, expanding soft-tissue hematomas. Laboratory tests revealed a coagulopathy consistent with plasma protein depletion following therapeutic plasma exchange, characterized by severe hypofibrinogenemia (1.35 g\/L) and a 50% reduction in total serum protein. TPE was permanently discontinued. The patient was successfully stabilized using aggressive fluid resuscitation, vasopressors, and fresh frozen plasma (FFP) transfusions, followed by maintenance therapy with rituximab. Conclusions: In conclusion, clinicians should remain vigilant that severe hyperviscosity syndrome can be driven by a polyclonal increase in immunoglobulins rather than just monoclonal entities; furthermore, managing this condition requires careful balancing of TPE efficacy against its potential to trigger profound depletion coagulopathy.<\/jats:p>","DOI":"10.3390\/reports9030207","type":"journal-article","created":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T08:19:02Z","timestamp":1782893942000},"page":"207","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Polyclonal Hyperviscosity Crisis and Severe Depletion Coagulopathy Induced by Therapeutic Plasma Exchange in Sj\u00f6gren\u2019s Syndrome: A Case Report and Therapeutic Dilemma"],"prefix":"10.3390","volume":"9","author":[{"given":"Gabriela","family":"Rybka","sequence":"first","affiliation":[{"name":"Clinical Department of Rheumatology, Immunology and Internal Medicine, University Hospital in Krak\u00f3w, Jakubowskiego 2, 30-688 Krak\u00f3w, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrzej","family":"Boryczko","sequence":"additional","affiliation":[{"name":"Clinical Department of Rheumatology, Immunology and Internal Medicine, University Hospital in Krak\u00f3w, Jakubowskiego 2, 30-688 Krak\u00f3w, Poland"},{"name":"Doctoral School of Medical and Health Sciences, Jagiellonian University Medical College, \u015aw. \u0141azarza 16, 31-530 Krak\u00f3w, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0321-3557","authenticated-orcid":false,"given":"Rados\u0142aw","family":"Dziedzic","sequence":"additional","affiliation":[{"name":"Clinical Department of Rheumatology, Immunology and Internal Medicine, University Hospital in Krak\u00f3w, Jakubowskiego 2, 30-688 Krak\u00f3w, Poland"},{"name":"Doctoral School of Medical and Health Sciences, Jagiellonian University Medical College, \u015aw. \u0141azarza 16, 31-530 Krak\u00f3w, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u0141ukasz","family":"Chmura","sequence":"additional","affiliation":[{"name":"Department of Pathomorphology, Faculty of Medicine, Jagiellonian University Medical College, Grzeg\u00f3rzecka 16, 31-531 Krak\u00f3w, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1013-2253","authenticated-orcid":false,"given":"Joanna","family":"Kosa\u0142ka-W\u0119giel","sequence":"additional","affiliation":[{"name":"Clinical Department of Rheumatology, Immunology and Internal Medicine, University Hospital in Krak\u00f3w, Jakubowskiego 2, 30-688 Krak\u00f3w, Poland"},{"name":"Department of Rheumatology and Immunology, Jagiellonian University Medical College, Jakubowskiego 2, 30-688 Krak\u00f3w, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,7,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1097\/BOR.0000000000000989","article-title":"Cryoglobulinemic vasculitis: A 2023 update","volume":"36","author":"Moretti","year":"2024","journal-title":"Curr. 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