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case_images: MRI Brain on Initial Presentation. Multiple MRI images reveal bilateral labyrinthine hemorrhages with intrinsic T1 hyperintensity ((A and B) arrows), more prominent on the left, representing blood products. Loss of normal signal suppression is noted throughout the inner ear on FLAIR image (C, arrow).
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case_images: MRI brain revealed bilateral small volume internal ear hemorrhage, Figure 1A-C.
case_images: Pre-Treatment Fundoscopic Evaluation and Optical Coherence Tomography. Patient's initial visual acuity of 20/150 OD and 20/100 OS correlates with initial fundoscopic exam showing evidence of venous occlusive disease with circumferential hemorrhages (arrow heads) and macular edema (arrows) on fundus exam ((A) OD; (B) OS). Initial OCT shows significant bilateral subretinal fluid (arrow heads) and bilateral intraretinal fluid (arrows), more prominent OS ((C) OD; (D) OS).
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case_images: Fundoscopic examination showed diffuse intraretinal hemorrhages, mild venous dilation and tortuosity, and serous macular detachments (SMD), Figure 2A and B. Initial optical coherence tomography (OCT) showed significant subretinal fluid bilateral and intraretinal fluid OS, Figure 2C and D.
case_images: Bone Marrow Biopsy and IHC Staining. Bone marrow biopsy revealed a diffuse and interstitial proliferation of small to medium lymphocytes and plasma cells (A, H&E x 10). IHC studies show that the lymphocytes were strongly and diffusely positive for CD20 (B, x 20); CD138 showed increased plasma cells (C, x 20); and CD79a confirmed the lymphoplasmacytic proliferation by highlighting both neoplastic B-lymphocytes and plasma cells (D, x 20). In addition, the lymphocytes and plasma cells are negative for kappa (E, x 20) but positive for lambda (F, x 20); hence confirmed lambda light chain restriction.
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case_images: Repeat bone marrow biopsy revealed a diffuse and interstitial proliferation of small to medium lymphocytes and plasma cells, Figure 3A. Immunohistochemistry (IHC) studies revealed the lymphoplasmacytic infiltrate was strongly and diffusely positive for CD20, CD79a, and CD138, Figure 3B-F.
case_images: Clinical Course as Per IgM Levels. Initial IgM levels were treated with 3 sessions of PLEX followed by initiation of RVD. Patient failed therapy on RVD as seen with spike in IgM levels to 6680 mg/dL requiring stabilization with 2 sessions of PLEX. The patient was then started on zanubrutinib, a BTKi, and was able to achieve a PR.
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case_images: A graph demonstrating pertinent IgM levels throughout her clinical course is shown in Figure 4. 
case_images: Post-Treatment Fundoscopic Evaluation, Optical Coherence Tomography, and Fluorescein Angiography. (A) (OD) and (B) (OS) were taken 7 months later and demonstrated significant fundoscopic improvement after plasma exchange, systemic therapy, and intravitreal bevacizumab. Her visual acuity improved to 20/50 OS; however, residual macular edema remains (arrows). After systemic treatment and intravitreal bevacizumab, the intraretinal and subretinal fluid improved but did not resolve on repeat OCT 7 months later and explains only partial visual recovery OS ((C) OD; (D) OS). These images suggest that the vision loss was primarily due to an exudative retinopathy such as WM-related SMD rather than CRVO as originally diagnosed. Recent FA shows no evidence of vascular leakage to explain the source of intra- and subretinal fluid ((E) OD; (F) OS).
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case_images: Serial fundoscopic examinations are shown with eventual resolution of the retinal hemorrhages (Figure 5A and B). Repeat OCT revealed minimal improvement in the bilateral subretinal fluid collections and intraretinal fluid OS despite intravitreal injections and systemic therapy (Figure 5C and D). Fluorescein angiography (FA) revealed no evidence of retinal vascular or pigment epithelial leakage to explain the etiology of the intra- and subretinal fluid (Figure 5E and F).
case_images: Initial and Post-Treatment Audiogram. (A) shows initial audiogram testing; red signifies right ear and blue the left ear. Word recognition was 0% when presented at 100 dB HL in the right ear and could not be tested in the left ear due to a lack of response. Right PTA was 62 dB HL, left ear could not be calculated. The right ear showed a moderate sloping to profound sensorineural hearing loss with very poor word understanding (0%). The left ear did not have any response to sound at the limits of testing. (B) shows 2-month repeat audiogram testing after plasma exchange and induction therapy. Word recognition was 53% when presented at 75 dB HL in the right ear and could not be tested in the left ear due to a lack of response. Right PTA was 52 dB HL, left ear could not be calculated. The right ear showed a moderate sloping to severe sensorineural hearing loss with poor word understanding ability (53%). The left ear did not have any response to sound at the limits of testing. The right ear improved an average of 10 dB PTA and showed partial recovery in word understanding ability. This significant improvement resulted in the patient being able to use amplification (hearing aids) successfully. The patient obtained BiCROS hearing aids to maximize her remaining auditory function.
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case_images: In terms of her auditory function, the initial testing revealed significant hearing loss in the right and profound deafness in the left ear (Figure 6A). Repeat audiology testing two months later revealed partial recovery in the right ear but persistent profound deafness in the left ear (Figure 6B).