The Spectrum of Ocular Manifestations in Patients with Waldenström’s Macroglobulinemia

ABSTRACT

Purpose

To investigate the ocular manifestations in 91 Waldenström’s macroglobulinemia (WM) patients.

Methods

Retrospective, cross-sectional, observational analysis.

Results

Ocular impairments, detected in 19 patients, included flame-shaped hemorrhages, venous sausaging, papilledema, macular detachments, or central retinal vein occlusion in 16 patients; paraproteinemic keratopathy in 2; and a CANOMAD syndrome in 1. Best-corrected visual acuity was ≥0.5 logMAR units in 11 of 38 eyes. Intraocular pressure was increased in seven eyes. Genetic analysis in seven patients showed a mutation in the MYD88 gene in six patients and a nonsense mutation in the CXCR4 gene in five patients. Plasmapheresis followed by chemotherapy with or without the addition of rituximab resulted in improvement or normalization of the ophthalmological findings in 15 patients.

Conclusion

The ocular manifestations of WM are protean and potentially sight threatening. Recent advances in genomic profiling and chemotherapy have remarkably improved the hematological and ophthalmological outcomes of these patients.

KEYWORDS:

• Hyperviscosity-related retinopathy
• immunochemotherapy
• macular detachment
• paraproteinemic keratopathy
• Waldenström’s macroglobulinemia

Acknowledgments

The authors have no relevant conflicts.

Authors’ contributions

RD, GA, AV and FD conceived and designed the study, had full access to all the data in the study, and take responsibility for the integrity of the data and the accuracy of the analysis. WL and TTK contributed to the study design and interpretation. RD and FD wrote the manuscript. ET and EK revised the manuscript for important intellectual content and supplied additional ocular images of Waldenström’s macroglobulinemia. DS, AM and RR retrieved and collected the data, and helped in their interpretation. All authors reviewed the manuscript, approved the draft submission, and accept responsibility for all aspects of this study.

Declaration of interest statement

The authors report no financial conflict of interest. The authors alone are responsible for the content and writing of the article.