{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,28]],"date-time":"2026-03-28T02:46:44Z","timestamp":1774666004547,"version":"3.50.1"},"reference-count":46,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,2,9]],"date-time":"2024-02-09T00:00:00Z","timestamp":1707436800000},"content-version":"vor","delay-in-days":8,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Clinical Apheresis"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Lipoprotein X (Lp\u2010X) is an abnormal lipoprotein found in multiple disease conditions, including liver dysfunction and cholestasis. High Lp\u2010X concentrations can interfere with some laboratory testing that may result in spurious results. The detection of Lp\u2010X can be challenging, and there is currently a lack of consensus regarding the management of Lp\u2010X other than treating the underlying disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Presentation<\/jats:title><jats:p>A 42\u2010year\u2010old female with Hodgkin's lymphoma treated with dexamethasone, high dose cytarabine and cisplatin and vanishing bile duct syndrome confirmed by liver biopsy presented with cholestasis, pseudohyponatremia (sodium, 113\u2009mmol\/L; reference range 136\u2013146\u2009mmL\/L; serum osmolality, 303\u2009mOsm\/kg), and hypercholesterolemia (&gt;\u20092800\u2009mg\/dL, reference range\u2009&lt;\u2009200\u2009mg\/dL). Lp\u2010X was confirmed by lipoprotein electrophoresis (EP). Although she did not manifest any specific signs or symptoms, therapeutic plasma exchange (TPE) was initiated based on laboratory findings of extreme hypercholesterolemia, spuriously abnormal serum sodium, and HDL values, and the potential for short\u2010 and long\u2010term sequelae such as hyperviscosity syndrome, xanthoma, and neuropathy. During the hospitalization, she was treated with four 1.0 plasma volume TPE over 6\u2009days using 5% albumin for replacement fluid. After the first TPE, total cholesterol (TC) decreased to 383\u2009mg\/dL and sodium was measured at 131\u2009mmol\/L. The patient was transitioned into outpatient maintenance TPE to eliminate the potential of Lp\u2010X reappearance while the underlying disease was treated. Serial follow\u2010up laboratory testing with lipoprotein EP showed the disappearance of Lp\u2010X after nine TPEs over a 10\u2010week period.<\/jats:p><\/jats:sec><jats:sec><jats:title>Literature Review<\/jats:title><jats:p>There are seven and four case reports of Lp\u2010X treated with TPE and lipoprotein apheresis (LA), respectively. While all previous case reports showed a reduction in TC levels, none had monitored the disappearance of Lp\u2010X after completing a course of therapeutic apheresis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Clinicians should have a heightened suspicion for the presence of abnormal Lp\u2010X in patients with cholestasis, hypercholesterolemia, and pseudohyponatremia. Once Lp\u2010X is confirmed by lipoprotein EP, TPE should be initiated to reduce TC level and remove abnormal Lp\u2010X. Most LA techniques are not expected to be beneficial since Lp\u2010X lacks apolipoprotein B. Therefore, we suggest that inpatient course of TPE be performed every other day until serum sodium, TC and HDL levels become normalized. Outpatient maintenance TPE may also be considered to keep Lp\u2010X levels low while the underlying disease is treated. Serum sodium, TC, and HDL levels should be monitored while on maintenance TPE.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jca.22105","type":"journal-article","created":{"date-parts":[[2024,2,9]],"date-time":"2024-02-09T12:13:29Z","timestamp":1707480809000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Use of therapeutic plasma exchange to remove lipoprotein X in a patient with vanishing bile duct syndrome presenting with cholestasis, pseudohyponatremia, and hypercholesterolemia: A case report and review of literature"],"prefix":"10.1002","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-9459-5296","authenticated-orcid":false,"given":"Yujung","family":"Jung","sequence":"first","affiliation":[{"name":"Bloodworks Northwest  Seattle Washington USA"},{"name":"Department of Laboratory Medicine and Pathology University of Washington  Seattle Washington 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