title: A Man with Paraneoplastic Retinopathy plus Small Fiber Polyneuropathy Associated with Waldenström Macroglobulinemia (Lymphoplasmacytic Lymphoma): Insights into Mechanisms.
abstract: OBJECTIVE
  To report a well-characterized Waldenström's macroglobulinemia (WM) case that provides insight into the mechanisms of two paraneoplastic complications -- cancer-associated retinopathy (CAR) and small fiber polyneuropathy (SFPN).
  
  
  METHODS
  Retrospective medical chart review.
  
  
  RESULTS
  A 58-year old man with WM developed vision loss and bilateral lower extremity pain. CAR was diagnosed by history, a depressed electroretinogram (ERG) and positive anti-retinal antibodies. SFPN diagnosis was based on abnormal autonomic nerve function testing and a distal-leg skin biopsy that demonstrated absent epidermal small-fiber innervation, IgM and complement deposition and microvasculopathy. Plasma exchange (PLEX) led to dramatic pain relief and subjective improvement in eye symptoms along with improvement of some ERG parameters. Repeat skin biopsy after treatment showed less microvascular abnormalities and decreased complement deposition.
  
  
  CONCLUSIONS
  The concurrence of CAR and SFPN in this patient suggest that both were complications of WM and their common response to PLEX suggests co-mediation by humoral factors that accessed target antigens through IgM-triggered, complement-mediated vascular damage.
authors.affiliation: a Department of Ophthalmology , Massachusetts Eye and Ear Infirmary, Harvard Medical School , Boston , Massachusetts , USA .
authors.forename: Yingna
authors.initials: Y
authors.lastname: Liu
authors.affiliation: b Department of Pathology and Laboratory Medicine , Weill Cornell Medical College of Cornell University , New York , USA .
authors.forename: Cynthia
authors.initials: C
authors.lastname: Magro
authors.affiliation: a Department of Ophthalmology , Massachusetts Eye and Ear Infirmary, Harvard Medical School , Boston , Massachusetts , USA .
authors.forename: John I
authors.initials: JI
authors.lastname: Loewenstein
authors.affiliation: c Department of Pathology , Blood Transfusion Service .
authors.forename: Robert S
authors.initials: RS
authors.lastname: Makar
authors.affiliation: c Department of Pathology , Blood Transfusion Service .
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Stowell
authors.affiliation: c Department of Pathology , Blood Transfusion Service .
authors.forename: Walter H
authors.initials: WH
authors.lastname: Dzik
authors.affiliation: d Department of Oncology , and.
authors.forename: Ephraim P
authors.initials: EP
authors.lastname: Hochberg
authors.affiliation: e Departments of Neurology and Pathology (Neuropathology) , Harvard Medical School, Massachusetts General Hospital , Boston , Massachusetts , USA.
authors.forename: Anne Louise
authors.initials: AL
authors.lastname: Oaklander
authors.affiliation: a Department of Ophthalmology , Massachusetts Eye and Ear Infirmary, Harvard Medical School , Boston , Massachusetts , USA .
authors.forename: Lucia
authors.initials: L
authors.lastname: Sobrin
mesh_terms: D003937:Diagnosis, Differential; D004596:Electroretinography; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D009412:Nerve Fibers / Q000473:pathology*; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000175:diagnosis; D010507:Periodicity; D011115:Polyneuropathies / Q000150:complications* / Q000175:diagnosis; D012160:Retina / Q000473:pathology*; D012164:Retinal Diseases / Q000150:complications* / Q000175:diagnosis; D012189:Retrospective Studies; D012867:Skin / Q000294:innervation / Q000473:pathology*; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000175:diagnosis
keywords: Autoimmune retinopathy; cancer-associated retinopathy; paraneoplastic retinopathy; plasmapheresis; rituximab