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Paraproteinemic Maculopathy in a 63-Year-Old Man

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 63-year-old man was seen with decreased vision in both eyes. He had been diagnosed as having type 2 diabetes for 10 years. His visual acuity was 20/40 OU. Fundus examination showed venous dilatation, retinal hemorrhages, hard exudates, and serous macular detachment (arrowheads) (Figure 1). Optical coherence tomography scans showed intraretinal fluid accumulation with serous macular detachment in both eyes. He was diagnosed as having nonproliferative diabetic retinopathy with macular edema in both eyes. He received 3 intravitreal bevacizumab injections at 4 weekly intervals and 2 intravitreal triamcinalone acetonide injections in both eyes, with minimal resolution of edema. Blood analysis revealed a low hemoglobin level of 8 g/dL, red blood cell count of 3.3 × 106/μL, white blood cell count of 3460/μL (15% neutrophils, 84% lymphocytes, and 1% monocytes), fasting blood glucose level of 120 mg/dL, normal lipid profile, serum urea nitrogen level of 45 mg/dL, and an increased serum creatinine level of 1.4 mg/dL (to convert hemoglobin to grams per liter, multiply by 10.0; red blood cell count to ×1012 per liter, multiply by 1.0; white blood cell count to ×109 per liter, multiply by 0.001; blood glucose to millimoles per liter, multiply by 0.0555; serum urea nitrogen to millimoles per liter, multiply by 0.357; and serum creatinine to micromoles per liter, multiply by 88.4).

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Paraproteinemic maculopathy

C. Do plasma electrophoresis and bone marrow analysis

This patient’s blood analysis revealed anemia, an increased white blood cell count with lymphocytosis, and an elevated serum creatinine level. All of these pointed to an underlying hyperviscosity syndrome secondary to a systemic disease. Nonresponse to antipermeability agents was also consistent with a nondiabetic etiology. Bone marrow study showed plasmacytosis (30%). Serum electrophoresis revealed an M-protein spike. A diagnosis of multiple myeloma with paraproteinemic maculopathy was made, and the patient was started chemotherapy. However, within weeks of starting the therapy, the patient had a cerebrovascular accident.

Paraproteinemia is a monoclonal gammopathy. It encompasses diseases like multiple myeloma, Waldenström macroglobulinemia, and cryoglobulinemia. Excess immunoglobulins in the blood lead to hyperviscosity.1 Multiple myeloma is a plasma cell dyscrasia characterized by high levels of a paraprotein called monoclonal protein, or M protein, in the blood and urine. Major organ systems involved in hyperviscosity syndromes include eye, heart (congestive heart failure), and brain (cerebrovascular accidents).

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Article Information

Corresponding Author: Pratyusha Ganne, MS, Aravind Eye Hospital, Cuddalore Road, Puducherry 605007, India (pratyusha080@gmail.com).

Published Online: May 10, 2018. doi:10.1001/jamaophthalmol.2017.6285

Conflict of Interest Disclosures: Both authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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4.
Berta  A, Beck  P, Mikita  J.  IgM paraprotein in the subretinal fluid of a patient with recurrent retinal detachment and Waldenström’s macroglobulinaemia.  Acta Med Hung. 1985;42(3-4):179-186.PubMedGoogle Scholar
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Brody  JM, Butrus  SI, Ashraf  MF, Rabinowitz  AI, Whitmore  PV.  Multiple myeloma presenting with bilateral exudative macular detachments.  Acta Ophthalmol Scand. 1995;73(1):81-82.PubMedGoogle ScholarCrossref
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Kyle  RA, Rajkumar  SV.  Treatment of multiple myeloma: a comprehensive review.  Clin Lymphoma Myeloma. 2009;9(4):278-288.PubMedGoogle ScholarCrossref
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Faiman  B, Bilotti  E, Mangan  PA, Rogers  K; IMF Nurse Leadership Board.  Steroid-associated side effects in patients with multiple myeloma: consensus statement of the IMF Nurse Leadership Board.  Clin J Oncol Nurs. 2008;12(3)(suppl):53-63.PubMedGoogle ScholarCrossref
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Fenicia  V, Balestrieri  M, Perdicchi  A, Maraone  G, Recupero  SM.  Intravitreal injection of dexamethasone implant in serous macular detachment associated with Waldenström’s disease.  Case Rep Ophthalmol. 2013;4(2):64-69.PubMedGoogle ScholarCrossref
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