TY - JOUR
T1 - WALDENSTRÖM'S MACROGLOBULINEMIA MASQUERADING AS BIRDSHOT CHORIORETINOPATHY
AU - Kim, Jane S.
AU - Jaworski, Laurence
AU - Patel-Donnelly, Dipti
AU - Nussenblatt, Robert B.
AU - Nida Sen, H.
N1 - Funding Information:
Supported by the National Eye Institute (NEI) Intramural Research Program and the National Institutes of Health (NIH) Medical Research Scholars Program, a public-private partnership supported jointly by the NIH and generous contributions to the Foundation for the NIH from Pfizer, Inc.
PY - 2017
Y1 - 2017
N2 - 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.
AB - 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.
KW - HLA-A29
KW - Waldenström's macroglobulinemia
KW - birdshot chorioretinopathy
KW - lymphoplasmacytic lymphoma
KW - macular edema
KW - masquerade syndrome
KW - rituximab
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U2 - 10.1097/ICB.0000000000000316
DO - 10.1097/ICB.0000000000000316
M3 - Article
C2 - 27124793
AN - SCOPUS:85017013396
SN - 1935-1089
VL - 11
SP - 152
EP - 155
JO - Retinal Cases and Brief Reports
JF - Retinal Cases and Brief Reports
IS - 2
ER -