Right orbital edema masquerading a hematologic malignancy

Abstract

Introduction: Multiple myeloma is caused by abnormal proliferation of plasma cells that affects more commonly African Americans. It classically presents with hypercalcemia, renal failure, anemia, and lytic bone lesions. The aim of this article is to present an unusual case of a 63-year-old African-American female with multiple myeloma who presented with worsening right-sided eye swelling for the past 3 weeks and to briefly review ophthalmologic manifestations of multiple myeloma.

Case description: Our patient's presentation was associated with a throbbing frontal headache, nasal congestion, malaise, and weight loss. Differential diagnosis on admission included giant cell arteritis, conjunctivitis, preseptal cellulitis, glaucoma, acute sinusitis, or cavernous sinus thrombosis. Extensive ophthalmologic evaluation did not show any intraocular abnormality. However, a magnetic resonance imaging of the brain showed hyperintense foci in the right frontal calvarium leading to the eye swelling. Further evaluation revealed pancytopenia, elevated protein levels, and inverse albumin-globulin ratio suggestive of a plasma cell dyscrasia. A skeletal survey revealed multiple osteolytic lesions. Serum and urine protein electrophoresis revealed elevated immunoglobulin G Kappa monoclonal gammopathy. Bone marrow biopsy demonstrated a hypercellular marrow comprised at least 70% mature appearing plasma cells staining positive for CD138. Chemotherapy with cyclophosphamide, bortezomib, and dexamethasone was initiated. After 2 months of chemotherapy, orbital swelling has resolved with decrease in M-spike, immunoglobulin G, and serum kappa light chains.

Conclusion: This case illustrates an unusual presentation of multiple myeloma which was eye swelling caused by bony infiltration in the calvarium. Although hematologic malignancies tend to have more specific signs and symptoms, they should be included in the differentials of unilateral orbital edema.

Keywords: Eye swelling; multiple myeloma; orbital edema.

Figure 1.

(a) Right eye swelling, (b) resolution of eye swelling after 2 months of chemotherapy, and (c) MRI of head and neck showing T2 hyperintensities in the calvarium.

Figure 2.

Bone marrow biopsy stained with hematoxylin and eosin showing a solid proliferation of mature appearing plasma cells. Left: objective 200×; right: objective 400×.

Figure 3.

Bone marrow biopsy immunohistochemical stain CD138 used to highlight plasma cells. Objective 400×.

Figure 4.

Flow cytometry showing plasma cells isolated with CD38 and CD138 antigens. They are kappa clonal and express CD56. Both markers indicate a malignancy.

Figure 5.

Fluorescence in situ hybridization (FISH) test was performed using dual color, dual fusion probes to detect the translocation between the CCND1 gene located at 11q (orange probe) and the IGH@ gene located at 14q (green probe). Signal that has joined orange and green color confirms presence of translocation (11;14)(q13.3;q32.3).