Case 1009 - A Female in Her 60s with Diplopia

Hematopathology

Contributed by Terri Jones, MD and Nidhi Aggarwal, MD
Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

CLINICAL HISTORY

The patient is a 60-70 year old female with a history of increased intraocular pressure felt to be due to ocular hypertension versus glaucoma and a selective laser trabeculoplasty of her left eye. She presented to her ophthalmologist for a routine intraocular pressure check. On examination, she was noted to have diplopia, proptosis, and limitation of elevation/abduction and no pain with orbital movement. A CT sinus/orbit was performed which showed an ill- defined mass within the left retrobulbar, superonasal soft tissue and involving the left lacrimal gland (see Fig 1). A lacrimal gland biopsy was performed which showed mild chronic dacryoadenitis, but ophthalmology was concerned that the mass was not adequately sampled and proceeded with a retrobulbar biopsy. This biopsy was received by Hematopathology in consultation without accompanying flow cytometric immunophenotypic studies.

LABORATORY AND MICROSCOPIC FINDINGS

Table 1. Complete blood count with differential

 The patient's CBC with differential at the time of biopsy is shown in Table 1.

Microscopic examination revealed extensive deposition of amorphous congophilic material that showed apple-green birefringence upon polarization, which is consistent with amyloid (see Figures 2, 3 and 4). There were also scattered B-cell aggregates with an occasional follicle with associated germinal center. The lymphoid aggregates stained positive for CD20, CD138, CD19, and negative for cyclin D1 and CD56, indicating the cells represented a plasmacytic neoplasm, likely a b-cell lymphoma with plasmacytic differentiation, see Figure 5. Additionally, the cells showed kappa restriction, see Figure 6.

FINAL DIAGNOSIS