title: Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease.
abstract: Light chain deposition disease (LCDD) is characterized by monotypic immunoglobulin depositions which will eventually lead to loss of organ function if left untreated. While the kidney is almost always affected, the presence and degree of LCDD in other organs vary. Ten to thirty percent of LCDD patients have underlying Multiple Myeloma (MM), yet outcome and prognostic markers in this particular patient group are still lacking. Here, we analyzed 69 patients with MM and biopsy proven LCDD and report on renal and extra-renal involvement and its impact on prognosis as well as renal response depending on hematologic response. Coexisting light chain diseases such as AL amyloid and cast nephropathy were found in 30% of patients; those with LCDD and concurrent amyloid tended to have shorter survival. Cardiac involvement by LCDD was seen in one-third of our patients and was associated with shorter overall survival; such patients also had a significantly higher risk of treatment-related mortality (TRM) after stem cell transplant (SCT) compared to LCDD patients without cardiac involvement. This study highlights that MM patients with LCDD present with different clinical features compared to previously reported LCDD cohorts. Rapid initiation of treatment is necessary to prevent progressive renal disease and worse outcome. Coexisting light chain diseases and cardiac involvement are more common than previously reported and confer worse clinical outcome, emphasizing the need for careful patient careful patient evaluation and treatment selection.
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Meera
authors.initials: M
authors.lastname: Mohan
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Amy
authors.initials: A
authors.lastname: Buros
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Pankaj
authors.initials: P
authors.lastname: Mathur
authors.affiliation: Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Neriman
authors.initials: N
authors.lastname: Gokden
authors.affiliation: Division for Nephrology, Department of Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Manisha
authors.initials: M
authors.lastname: Singh
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Sandra
authors.identifier: http://orcid.org/0000-0003-0703-2518
authors.initials: S
authors.lastname: Susanibar
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Jorge
authors.initials: J
authors.lastname: Jo Kamimoto
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Shadiqul
authors.initials: S
authors.lastname: Hoque
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Muthukumar
authors.initials: M
authors.lastname: Radhakrishnan
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Aasiya
authors.initials: A
authors.lastname: Matin
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Davis
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Monica
authors.initials: M
authors.lastname: Grazziutti
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Sharmilan
authors.initials: S
authors.lastname: Thanendrarajan
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Frits
authors.initials: F
authors.lastname: van Rhee
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Maurizio
authors.identifier: http://orcid.org/0000-0002-1720-3854
authors.initials: M
authors.lastname: Zangari
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Faith
authors.initials: F
authors.lastname: Davies
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Gareth
authors.initials: G
authors.lastname: Morgan
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Joshua
authors.initials: J
authors.lastname: Epstein
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Bart
authors.initials: B
authors.lastname: Barlogie
authors.affiliation: Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Carolina
authors.identifier: http://orcid.org/0000-0002-2699-1741
authors.initials: C
authors.lastname: Schinke
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers; D001706:Biopsy; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007674:Kidney Diseases / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism* / Q000628:therapy; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D011379:Prognosis; D066263:Protein Aggregation, Pathological; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D007147:Immunoglobulin Light Chains