PMID- 27124793 OWN - NLM STAT- MEDLINE DCOM- 20170918 LR - 20240109 IS - 1937-1578 (Electronic) IS - 1935-1089 (Print) IS - 1935-1089 (Linking) VI - 11 IP - 2 DP - 2017 Spring TI - WALDENSTROM'S MACROGLOBULINEMIA MASQUERADING AS BIRDSHOT CHORIORETINOPATHY. PG - 152-155 LID - 10.1097/ICB.0000000000000316 [doi] AB - PURPOSE: To describe Waldenstrom'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. FAU - Kim, Jane S AU - Kim JS AD - *Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, MD; daggerMedical Research Scholars Program, National Institutes of Health, Bethesda, MD; double daggerSchool of Medicine, University of California, San Diego, La Jolla, CA; section signIntramural Research Program, National Eye Institute, National Institutes of Health, Bethesda, MD; and paragraph signVirginia Cancer Specialists, Fairfax, VA. FAU - Jaworski, Laurence AU - Jaworski L FAU - Patel-Donnelly, Dipti AU - Patel-Donnelly D FAU - Nussenblatt, Robert B AU - Nussenblatt RB FAU - Sen, H Nida AU - Sen HN LA - eng GR - Z99 EY999999/Intramural NIH HHS/United States PT - Case Reports PT - Journal Article PL - United States TA - Retin Cases Brief Rep JT - Retinal cases & brief reports JID - 101298744 SB - IM EIN - Retin Cases Brief Rep. 2020 Summer;14(3):e3. PMID: 29621043 MH - Birdshot Chorioretinopathy MH - Chorioretinitis/*diagnosis MH - Diagnosis, Differential MH - Humans MH - Male MH - Middle Aged MH - Multimodal Imaging MH - Paraneoplastic Syndromes, Ocular/*diagnosis MH - Waldenstrom Macroglobulinemia/*diagnosis PMC - PMC5083234 MID - NIHMS764808 COIS- Disclosures: The authors declare that they have no conflicts of interest to disclose. EDAT- 2016/04/29 06:00 MHDA- 2017/09/19 06:00 PMCR- 2018/04/01 CRDT- 2016/04/29 06:00 PHST- 2016/04/29 06:00 [pubmed] PHST- 2017/09/19 06:00 [medline] PHST- 2016/04/29 06:00 [entrez] PHST- 2018/04/01 00:00 [pmc-release] AID - 10.1097/ICB.0000000000000316 [doi] PST - ppublish SO - Retin Cases Brief Rep. 2017 Spring;11(2):152-155. doi: 10.1097/ICB.0000000000000316.