Ocular complications of plasma cell dyscrasias

Abstract

Plasma cell dyscrasias are a wide range of severe monoclonal gammopathies caused by pre-malignant or malignant plasma cells that over-secrete an abnormal monoclonal antibody. These disorders are associated with various systemic findings, including ophthalmological disorders. A search of PubMed, EMBASE, Scopus and Cochrane databases was performed in March 2021 to examine evidence pertaining to ocular complications in patients diagnosed with plasma cell dyscrasias. This review outlines the ocular complications associated with smoldering multiple myeloma and monoclonal gammopathy of undetermined significance, plasmacytomas, multiple myeloma, Waldenström's macroglobulinemia, systemic amyloidosis, Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes (POEMS) syndrome, and cryoglobulinemia. Although, the pathological mechanisms are not completely elucidated yet, wide-ranging ocular presentations have been identified over the years, evolving both the anterior and posterior segments of the eye. Moreover, the presenting symptoms also help in early diagnosis in asymptomatic patients. Therefore, it is imperative for the treating ophthalmologist and oncologist to maintain a high clinical suspicion for identifying the ophthalmological signs and diagnosing the underlying disease, preventing its progression through efficacious treatment strategies.

Keywords: CORNEA / EXTERNAL DISEASE; GLAUCOMA; RETINA; Retinal degenerations associated with systemic disease; corneal degenerations.

Figure 1.

The figure shows fundus photograph and fluorescein angiography (fa) of a 62-year-old female patient with monoclonal gammopathy of undetermined significance (MGUS). There is presence of optic disc hyperemia on fundus photograph (a), with a dull foveal reflex suggestive of central macular edema. The FA shows corresponding leakage in the optic nerve and the macula, along with some leakage from the vessels in the early (b) and late phase (c).

Figure 2.

The figure shows a patient with multiple myeloma associated paraproteinemia (66-year-old Asian Indian female). The fundus photograph shows central area of diffuse orange area suggestive of retinal atrophy and visible underlying choroid, surrounded by a ring of lightly pigmented area, followed by a dark ring (in a target pattern) (a and b). The corresponding fundus autofluorescence image (FAF) also shows a ring pattern with a large central atrophy of retinal pigment epithelium (RPE) with hyper-autofluorescent outer ring.

Figure 3.

The figure shows the same patient as in figure 2 with positive findings on fluorescein angiography (FA) and optical coherence tomography (OCT). FA shows central hyperfluorescence in the macular region which is symmetrical in both eyes (a and b). On OCT, there is presence of hyper-reflective foci in the outer retina along with disruption of the ellipsoid zone, interdigitation zone, and external limiting membrane. Thinning of the retinal pigment epithelium is suggestive of atrophy (c and d).

Figure 4.

Optical coherence tomography angiography (OCTA) of the same patient as in figure 2. The right eye is represented in panels a and b, and left eye is panels c and d. The superficial retinal plexus shows largely preserved superficial capillary plexus and no evidence of signal loss on corresponding structural en face image (a and c). However, the deep retinal vascular plexus shows distinct well-defined loss of deep retinal capillaries (b and d) suggestive of deep retinal ischemia.