Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab

Purpose: To describe the clinical course and management of a patient with bilateral retinal vasculitis associated with cold agglutinin disease (CAD) treated with obinutuzumab and infliximab. Observations: A 69-year-old Hispanic woman was referred to a tertiary Uveitis Clinic with progressively worse...

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Main Authors: Ngoc Trong Tuong Than, Çigdem Yaşar, Brandon Huy Pham, Brandon Chau Lam, Huy Luong Doan, Amir Akhavanrezayat, Muhammad Sohail Halim, David Joseph Iberri, Doan Luong Hien, Quan Dong Nguyen
Format: Article
Language:English
Published: Elsevier 2022-12-01
Series:American Journal of Ophthalmology Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2451993622004984
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author Ngoc Trong Tuong Than
Çigdem Yaşar
Brandon Huy Pham
Brandon Chau Lam
Huy Luong Doan
Amir Akhavanrezayat
Muhammad Sohail Halim
David Joseph Iberri
Doan Luong Hien
Quan Dong Nguyen
author_facet Ngoc Trong Tuong Than
Çigdem Yaşar
Brandon Huy Pham
Brandon Chau Lam
Huy Luong Doan
Amir Akhavanrezayat
Muhammad Sohail Halim
David Joseph Iberri
Doan Luong Hien
Quan Dong Nguyen
author_sort Ngoc Trong Tuong Than
collection DOAJ
description Purpose: To describe the clinical course and management of a patient with bilateral retinal vasculitis associated with cold agglutinin disease (CAD) treated with obinutuzumab and infliximab. Observations: A 69-year-old Hispanic woman was referred to a tertiary Uveitis Clinic with progressively worsening blurry vision, right eye (OD) worse than left eye (OS). Past ocular history was significant for epiretinal membranes in both eyes (OU). Past medical history was notable for non-specific joint disease, primarily affecting her knees bilaterally, and pulmonary symptoms (e.g., dyspnea, productive cough) of unclear etiologies one year before presentation. She had been evaluated by rheumatologists and pulmonologists and was placed on low doses of prednisone and methotrexate. Upon examination, her visual acuity was 20/40 in OD and 20/25 in OS. Anterior segment exam was unremarkable with no cell or flare in OU. Dilated fundus examination was notable for 0.5+ vitreous haze in OU and mild vessel attenuation in OU. Wide-angle fluorescein angiography (FA) revealed mild bilateral periphery peri-vasculature leakage in OU. Initial blood evaluations revealed decreased hematocrit, and positive anti-nuclear antibody. Her peripheral smear disclosed 3+ agglutination. She was initially treated with mycophenolate mofetil 1000 mg twice daily and prednisone 20 mg then referred to hematology. Further work up revealed high-titer cold agglutinin and positive thermal amplitude screen at 30 °C. Bone marrow examination demonstrated a chronic lymphocytic leukemia (CLL)-like monoclonal B-cell lymphocytosis. Anti-CD20 monoclonal antibody therapy with obinutuzumab was started in an effort to treat the underlying CLL clone and address the associated ocular vasculitis related to CAD. Three months later, after eight cycles of obinutuzumab, the patient's best- corrected visual acuity (BCVA) continued to be stable at 20/30 in OD and 20/20 in OS. However, FA showed persistent diffuse perivascular leakage. Intravenous infliximab with concurrent intravenous methylprednisolone infusions were started. After two cycles of treatment, FA showed significantly improved perivascular leakage. Visual acuity remained stable at 20/25 in OU. Conclusions and importance: Ocular involvement in CAD is rare. The index case is the first report of retinal vasculitis in a patient with CAD. Our report not only describes the unique course of CAD-related retinal vasculitis, but also introduces and underscores a successful therapeutic plan.
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spelling doaj.art-606f16a4f4134cbca5cd3a43dd4dea542022-12-22T04:36:18ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362022-12-0128101752Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximabNgoc Trong Tuong Than0Çigdem Yaşar1Brandon Huy Pham2Brandon Chau Lam3Huy Luong Doan4Amir Akhavanrezayat5Muhammad Sohail Halim6David Joseph Iberri7Doan Luong Hien8Quan Dong Nguyen9Byers Eye Institute, Stanford University, Palo Alto, CA, USA; Pham Ngoc Thach University of Medicine, Saigon, Viet NamByers Eye Institute, Stanford University, Palo Alto, CA, USAByers Eye Institute, Stanford University, Palo Alto, CA, USAByers Eye Institute, Stanford University, Palo Alto, CA, USA; University of Texas Health Science Center, San Antonio, TX, USAKansas City University of Medicine and Biosciences, Kansas City, MO, USAByers Eye Institute, Stanford University, Palo Alto, CA, USAByers Eye Institute, Stanford University, Palo Alto, CA, USA; Ocular Imaging Research and Reading Center (OIRRC), Sunnyvale, CA, USADivision of Hematology-Oncology, Department of Medicine, Stanford University, Palo Alto, CA, USAByers Eye Institute, Stanford University, Palo Alto, CA, USA; Pham Ngoc Thach University of Medicine, Saigon, Viet NamByers Eye Institute, Stanford University, Palo Alto, CA, USA; Corresponding author. Spencer Center for Vision Research, Byers Eye Institute at Stanford University, 2452 Watson Court, Suite 200, Palo Alto, CA, 94303, USA.Purpose: To describe the clinical course and management of a patient with bilateral retinal vasculitis associated with cold agglutinin disease (CAD) treated with obinutuzumab and infliximab. Observations: A 69-year-old Hispanic woman was referred to a tertiary Uveitis Clinic with progressively worsening blurry vision, right eye (OD) worse than left eye (OS). Past ocular history was significant for epiretinal membranes in both eyes (OU). Past medical history was notable for non-specific joint disease, primarily affecting her knees bilaterally, and pulmonary symptoms (e.g., dyspnea, productive cough) of unclear etiologies one year before presentation. She had been evaluated by rheumatologists and pulmonologists and was placed on low doses of prednisone and methotrexate. Upon examination, her visual acuity was 20/40 in OD and 20/25 in OS. Anterior segment exam was unremarkable with no cell or flare in OU. Dilated fundus examination was notable for 0.5+ vitreous haze in OU and mild vessel attenuation in OU. Wide-angle fluorescein angiography (FA) revealed mild bilateral periphery peri-vasculature leakage in OU. Initial blood evaluations revealed decreased hematocrit, and positive anti-nuclear antibody. Her peripheral smear disclosed 3+ agglutination. She was initially treated with mycophenolate mofetil 1000 mg twice daily and prednisone 20 mg then referred to hematology. Further work up revealed high-titer cold agglutinin and positive thermal amplitude screen at 30 °C. Bone marrow examination demonstrated a chronic lymphocytic leukemia (CLL)-like monoclonal B-cell lymphocytosis. Anti-CD20 monoclonal antibody therapy with obinutuzumab was started in an effort to treat the underlying CLL clone and address the associated ocular vasculitis related to CAD. Three months later, after eight cycles of obinutuzumab, the patient's best- corrected visual acuity (BCVA) continued to be stable at 20/30 in OD and 20/20 in OS. However, FA showed persistent diffuse perivascular leakage. Intravenous infliximab with concurrent intravenous methylprednisolone infusions were started. After two cycles of treatment, FA showed significantly improved perivascular leakage. Visual acuity remained stable at 20/25 in OU. Conclusions and importance: Ocular involvement in CAD is rare. The index case is the first report of retinal vasculitis in a patient with CAD. Our report not only describes the unique course of CAD-related retinal vasculitis, but also introduces and underscores a successful therapeutic plan.http://www.sciencedirect.com/science/article/pii/S2451993622004984Cold agglutinin diseaseRetinal vasculitisAnti-CD20ObinutuzumabInfliximabTumor necrosis factor-alpha
spellingShingle Ngoc Trong Tuong Than
Çigdem Yaşar
Brandon Huy Pham
Brandon Chau Lam
Huy Luong Doan
Amir Akhavanrezayat
Muhammad Sohail Halim
David Joseph Iberri
Doan Luong Hien
Quan Dong Nguyen
Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab
American Journal of Ophthalmology Case Reports
Cold agglutinin disease
Retinal vasculitis
Anti-CD20
Obinutuzumab
Infliximab
Tumor necrosis factor-alpha
title Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab
title_full Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab
title_fullStr Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab
title_full_unstemmed Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab
title_short Bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab
title_sort bilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab
topic Cold agglutinin disease
Retinal vasculitis
Anti-CD20
Obinutuzumab
Infliximab
Tumor necrosis factor-alpha
url http://www.sciencedirect.com/science/article/pii/S2451993622004984
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