title: Primary sclerosing cholangitis complicating plasma cell dyscrasia (PCD): remission of PCD following liver transplant.
abstract: We report the first case of primary sclerosing cholangitis (PSC) complicated with plasma cell dyscrasia (PCD) in which liver transplant resulted in unexpected therapeutic benefit of PCD. A 61-year-old man with 12 yr history of PSC presented with a monoclonal gammopathy of undetermined significance (MGUS) with an IgG level of 3400 mg/dL. It was stable initially for 3 yr but progressed to features consistent with multiple myeloma (MM): IgG rose to 5290 mg/dL along with development of terminal stage of liver failure. Liver transplant was performed in desperation. Unexpectedly, MM underwent clinical remission following transplant. At 3 and 14 months following transplant, IgG stayed below 2080 mg/dL and he was able to return to full-time employment. This case may suggest that chronic antigenic stimulation from cirrhotic liver contributed to MGUS and subsequent transformation to MM. Liver transplant eliminated chronic antigenic stimulation, apparently leading to remission of MM. Since PCD is often associated with other chronic liver diseases, similar benefit may accrue to a range of patients with chronic liver diseases complicating PCD.
authors.affiliation: Division of Hematology/Oncology, University of Miami School of Medicine, Miami, FL 33136, USA.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Fontana
authors.forename: Yeon S
authors.initials: YS
authors.lastname: Ahn
authors.forename: Lawrence H
authors.initials: LH
authors.lastname: Horstman
authors.forename: Andreas G
authors.initials: AG
authors.lastname: Tzakis
authors.forename: Christopher B
authors.initials: CB
authors.lastname: O'Brien
mesh_terms: D015209:Cholangitis, Sclerosing / Q000150:complications*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications / Q000601:surgery*; D012074:Remission Induction
chemical_list: D007074:Immunoglobulin G