Waldenström's Macroglobulinemia Masquerading as Birdshot Chorioretinopathy

Abstract

Purpose To describe Waldenström’s macroglobulinemia (WM) as a masquerade syndrome. Method Case report. Results A 59-year-old human leukocyte antigen (HLA)-A29-negative Caucasian male presented with a seven-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 work-up 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 work-up 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.

Orbital involvement in Waldenstrom macroglobulinaemia: a multidisciplinary approach

“…Lymphomatous involvement of the ocular or periocular structures is a rare event found in 1 to 2% of NHL [2] with just a small number of reports to date in WM. These reports indicate that WM can involve the lacrimal glands [3], the retina [4], choroidal tissue [5] and extra-ocular muscles [1] as was present in our case. The optimal management of orbital involvement in WM is not well established; however, like the case reported by Adiga et al, in our case chemo-immunotherapy was the backbone of treatment.…”

Atypical White Dot Syndrome in an Older Man

“…Waldenström macroglobulinemia can masquerade as birdshot chorioretinopathy, idiopathic central serous retinopathy, or more common ocular diseases, such as diabetic or hypertensive retinopathy . This case highlights the importance of also considering malignancy when faced with a case of atypical chorioretinal lesions in an older patient.…”

“…Hyperviscosity symptoms develop in only 15% to 17% of patients; these may include "B symptoms," neurologic disturbances, and vascular abnormalities, including vertigo, epistaxis, gastrointestinal hemorrhage, and congestive heart failure. [1][2][3] Asymp-tomatic patients with WM can be monitored without treatment given its slow progression. 3 Ocular findings occur at varying levels of IgM.…”

Vitreous haemorrhage as the first presentation of undiagnosed Waldenstrom macroglobulinemia

“…In contrast to NHL in general, its ocular involvements are less well reported. There are few studies reporting conjunctival involvement [8] , the superior rectus muscle [10] , bilateral central retinal vein occlusions [11][12] , birdshot chorioretinopathy [13] , adnexal mass involving extraocular muscles [14] , and serous retinal detachment [15] . However, none of the cases report such dramatic, sudden reduction in vision by vitreous haemorrhage as a clinical feature nor as a presenting complaint of undiagnosed WM.…”