title: The Hematologic Definition of Monoclonal Gammopathy of Undetermined Significance in Relation to Paraproteinemic Keratopathy (An American Ophthalmological Society Thesis).
abstract: To determine if paraproteinemic keratopathy (PPK) in the setting of monoclonal gammopathy of undetermined significance (MGUS) causes distinct patterns of corneal opacification that can be distinguished from hereditary, immunologic, or inflammatory causes.
  
  
  
  A retrospective, interventional study of patients showed distinct bilateral opacity patterns of the cornea at the eye clinics of Hanau, Mainz, Helsinki, Marburg, and Berlin between 1993 and 2015. Data on patient characteristics and clinical features on ophthalmic examination were collected, and serum protein profiles were evaluated. A literature review and analysis of all published studies of MGUS with PPK is also presented.
  
  
  
  The largest group of patients diagnosed with MGUS-induced PPK is analyzed in this study. We studied 22 eyes of 11 patients (6 male, aged 43 to 65, mean age 54; 5 female, aged 49 to 76, mean age 61) with distinct corneal opacities and visual impairment who were first suspected of having hereditary, inflammatory, or immunologic corneal entities. Subsequently, serum protein electrophoresis revealed MGUS to be the cause of the PPK. Literature review revealed 72 patients with bilateral PPK (34 male, mean age 57; 38 female, mean age 58) in 51 studies of MGUS published from 1934 to 2015 and disclosed six additional corneal opacity patterns.
  
  
  
  This thesis shows that MGUS is not always an asymptomatic disorder, in contrast to the hematologic definition, which has no hint of PPK. The MGUS-induced PPK can mimic many other diseases of the anterior layer of the eye. A new clinical classification for PPK in MGUS is proposed.
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Walter
authors.initials: W
authors.lastname: Lisch
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Joanna
authors.initials: J
authors.lastname: Wasielica-Poslednik
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Tero
authors.initials: T
authors.lastname: Kivelä
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Ursula
authors.initials: U
authors.lastname: Schlötzer-Schrehardt
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Jens M
authors.initials: JM
authors.lastname: Rohrbach
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Walter
authors.initials: W
authors.lastname: Sekundo
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Uwe
authors.initials: U
authors.lastname: Pleyer
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Christina
authors.initials: C
authors.lastname: Lisch
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Alexander
authors.initials: A
authors.lastname: Desuki
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Heidi
authors.initials: H
authors.lastname: Rossmann
authors.affiliation: Department of Ophthalmology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany (Dr W. Lisch, Dr Wasielica-Poslednik); the Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Kivelä); the Department of Ophthalmology, University of Erlangen-Nürnberg, Erlangen, Germany (Dr Schlötzer-Schrehardt); the Department of Ophthalmology, Eberhard-Karls University of Tübingen, Tübingen, Germany (Dr Rohrbach); the Department of Ophthalmology, University of Marburg, Marburg, Germany (Dr Sekundo); the Department of Ophthalmology, Campus Virchow-Klinikum, Charité Universitaetsmedizin Berlin, Berlin, Germany (Dr Pleyer); the private practice of ophthalmology Hanau, Hanau, Germany (Dr C. Lisch); the Department of Internal Medicine III, Johannes Gutenberg University Mainz, Mainz, Germany (Dr Desuki); the Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany (Dr Rossmann); and the Department of Ophthalmology, Louisiana State University Health Sciences Center, School of Medicine, New Orleans, Louisiana (Dr Weiss).
authors.forename: Jayne S
authors.initials: JS
authors.lastname: Weiss
mesh_terms: D000328:Adult; D000368:Aged; D003315:Cornea / Q000000981:diagnostic imaging*; D003316:Corneal Diseases / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D009885:Ophthalmology; D010265:Paraproteinemias / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D012189:Retrospective Studies; D012955:Societies, Medical; D014481:United States