WALDENSTRÖM'S MACROGLOBULINEMIA MASQUERADING AS BIRDSHOT CHORIORETINOPATHY

Jane S. Kim, Laurence Jaworski, Dipti Patel-Donnelly, Robert B. Nussenblatt, H. Nida Sen

Research output: Contribution to journalArticle

Abstract

Purpose:To describe Waldenström's macroglobulinemia (WM) as a masquerade syndrome.Methods:Case report.Results:A 59-year-old human leukocyte antigen (HLA)-A29-negative white male presented with a 7-year history of floaters, progressive vision loss, and poor contrast sensitivity along with choroidal lesions suggestive of birdshot chorioretinopathy (BCR). Fluorescein angiography, fundus autofluorescence, and indocyanine green angiography showed multiple areas of hyperfluorescence and hypofluorescence. Electroretinography showed reduced cone and rod responses. Comprehensive workup for infectious, paraneoplastic, and other causes including sarcoidosis was negative at the time. The patient was treated with multiple immunomodulatory agents without any significant improvement. Two years after initial presentation, the patient developed normocytic anemia and high levels of inflammatory markers. Further workup yielded a diagnosis of WM. His choroidal lesions were significantly reduced after treatment with rituximab and bendamustine.Conclusion:We report a case of WM masquerading as BCR. Other indolent diseases should be considered in the differential diagnosis for HLA-A29-negative patients presenting with birdshot-like lesions, especially if they are clinically unresponsive to multiple systemic immunosuppressive agents.

Original languageEnglish (US)
Pages (from-to)152-155
Number of pages4
JournalRetinal Cases and Brief Reports
Volume11
Issue number2
DOIs
StatePublished - Jan 1 2017
Externally publishedYes

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Waldenstrom Macroglobulinemia
N,N-dimethyl-3,3-diphenyl-1-methylallylamine
HLA Antigens
Electroretinography
Vertebrate Photoreceptor Cells
Contrast Sensitivity
Indocyanine Green
Fluorescein Angiography
Immunosuppressive Agents
Sarcoidosis
Anemia
Angiography
Differential Diagnosis
Birdshot chorioretinopathy
Therapeutics

Keywords

  • birdshot chorioretinopathy
  • HLA-A29
  • lymphoplasmacytic lymphoma
  • macular edema
  • masquerade syndrome
  • rituximab
  • Waldenström's macroglobulinemia

ASJC Scopus subject areas

  • Ophthalmology

Cite this

WALDENSTRÖM'S MACROGLOBULINEMIA MASQUERADING AS BIRDSHOT CHORIORETINOPATHY. / Kim, Jane S.; Jaworski, Laurence; Patel-Donnelly, Dipti; Nussenblatt, Robert B.; Nida Sen, H.

In: Retinal Cases and Brief Reports, Vol. 11, No. 2, 01.01.2017, p. 152-155.

Research output: Contribution to journalArticle

Kim, Jane S. ; Jaworski, Laurence ; Patel-Donnelly, Dipti ; Nussenblatt, Robert B. ; Nida Sen, H. / WALDENSTRÖM'S MACROGLOBULINEMIA MASQUERADING AS BIRDSHOT CHORIORETINOPATHY. In: Retinal Cases and Brief Reports. 2017 ; Vol. 11, No. 2. pp. 152-155.
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