CASE REPORT
Epibulbar Plasmacytoma Masquerading as Subconjunctival
Hemorrhage in a Patient With Multiple Myeloma
Amanda Bradley, BS,* Amy Estes, MD,*† Lane Ulrich, MD,*† Dilip Thomas, MD,*† and David Gay, MD*†
Purpose: We report a 75-year-old woman with a history of multiple
myeloma immunoglobulin D (IgD) variant, who presented with an
epibulbar plasmacytoma masquerading as a subconjunctival hemorrhage.
Methods: Magnetic resonance imaging of the brain and orbits with
and without contrast was obtained and surgical biopsy of the
subconjunctival lesion was performed; histopathology confirmed the
diagnosis of plasmacytoma.
Results: Subconjunctival biopsy revealed a plasma cell neoplasm
infiltrate in the episcleral layer. The subconjunctival biopsy stained
positive for CD138 and lambda-immunohistochemistry in the majority
of plasma cells. Histologic findings were consistent with involvement
by known IgD plasma cell myeloma where previous bone marrow
biopsy demonstrated myeloma cells which stained monoclonally for
IgD-lambda light chains.
Conclusions: Although plasma cell neoplasms seldom present with
ocular manifestations, it is crucial to recognize that these tumors may
be associated with multiple myeloma. In patients with known multiple
myeloma who present with subconjunctival hemorrhage, close follow-
up is highly recommended, as this may be the initial presentation of an
ocular plasmacytoma. Although a plasmacytoma is a rare subcon-
junctival lesion, it should not be immediately excluded from the
differential diagnosis of such lesions.
Key Words: multiple myeloma, plasmacytoma, subconjunctival
hemorrhage
(Cornea 2017;36:249–251)
M ultiple myeloma is a treatable yet incurable malignancy,
which presents as monoclonal proliferation of abnormal
plasma cells, resulting in overproduction of a clone of
immunoglobulins. This plasma cell dyscrasia accounts for
approximately 10% of all hematologic malignances. The age-
adjusted incidence of multiple myeloma is 6.56 cases per
100,000 persons per year in the United States according to the
National Cancer Institute’s Surveillance Epidemiology and
End Result (SEER) data released in 2012.1 Multiple myeloma
Received for publication July 8, 2016; revision received September 19, 2016;
accepted September 20, 2016. Published online ahead of print November
16, 2016.
From the *Medical College of Georgia, Augusta University, Augusta, GA; and
†Department of Ophthalmology, Augusta University, Augusta, GA.
The authors have no funding or conflicts of interest to disclose.
Reprints: Amy Estes, MD, Department of Ophthalmology, Augusta Univer-
sity, 1120 15th St, Augusta, GA 30912 (e-mail: aestes@augusta.edu).
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Cornea  Volume 36, Number 2, February 2017
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is conventionally a disease of older adults, with the median
age of diagnosis being 66 years. African Americans are twice
as likely as whites to be affected by this disease.
Multiple myeloma is characterized by proliferation of
plasma cells in the bone marrow, which typically results in
widespread skeletal destruction as a result of osteolytic
lesions and associated pathologic fractures. Bone pain is
the most common clinical presentation; however, other
hallmarks of the disease include anemia, recurrent infec-
tions, hypercalcemia, spinal cord compression, and renal
insufficiency.2
Although rare, multiple myeloma may manifest with
involvement in virtually any ocular structure. A few of the
commonly reported ocular findings include orbital plasmacy-
tomas, ciliary body cysts, exudative macular detachment,
copper deposition in the cornea, retinal hemorrhages, and
cotton wool spots.3 Ocular pathology may occur as a result of
direct infiltration of malignant plasma cells, displacement of
ocular tissues by plasmacytomas, or hematologic derange-
ments with increased plasma viscosity or protein deposition in
tissues. To the best of our knowledge, this is the only case of
subconjunctival plasmacytoma initially disguised as
subconjunctival hemorrhage.
CASE REPORT
A 75-year-old woman with known refractory immunoglobulin D
(IgD) multiple myeloma was referred to the Ophthalmology Depart-
ment at Augusta University for evaluation of new-onset subconjunctival
hemorrhage. She reported mildly blurred vision with bulging of her left
eye and associated redness. She was receiving chemotherapy and had
recently had administered warfarin. She denied any recent trauma or
excessive rubbing. Slit-lamp examination of the conjunctiva revealed
diffuse subconjunctival hemorrhage, most prominent at the nasal bulbar
conjunctiva OS, resulting in delle formation. She received conservative
therapy with erythromycin ointment and artificial tears. At follow-up,
ocular examination of the conjunctiva and the sclera demonstrated
resolving subconjunctival hemorrhage. Evaluation of the cornea was
significant for resolution of the delle.
Four months later, the patient returned with complaints of
a 2-week history of recurrent subconjunctival hemorrhage in the
same location as the previous hemorrhage OS. Examination of the
conjunctiva and the sclera of her left eye demonstrated elevation of
the nasal bulbar conjunctiva with an erythematous and cystic
subconjunctival mass present in the location of the previous
subconjunctival hemorrhage (Fig. 1). A foamy epithelial lesion,
continuous with a subconjunctival mass extending 2 mm onto the
cornea, was noted on inspection. The remainder of her anterior
segment was unremarkable.
Although atypical in size and location, the initial clinical
concern was for a plasmacytoma, and magnetic resonance imaging
www.corneajrnl.com | 249
Bradley et al
FIGURE 1. Clinical appearance of
a subconjunctival mass on return
appointment in January 2016.
(MRI) was performed to rule out orbital involvement. MRI with
and without contrast of the brain and orbits showed a left medial
canthal episcleral soft tissue mass measuring 7.4 · 10.1 ·
14.6 mm in AP, transverse, and vertical diameters on axial
T2-weighted series (Fig. 2). The lesion demonstrated predomi-
nantly hypointense behavior with positive diffusion signal
abnormality concerning for high cellular packing. MRI demon-
strated mild elevation of the medial left eyelid due to the mass
molding itself to the junction of the left medial canthal tendon and
scleral surface. Underlying scleral and medial canthal structures,
including the anterior attachment of the medial rectus, appeared
normal and otherwise preserved.
A unilateral epibulbar subconjunctival neoplasm represent-
ing an ocular plasmacytoma was suspected. Excisional biopsy of
the lesion was performed. Histopathologic analysis was consistent
with a plasma cell neoplasm. Immunohistochemical stains demon-
strated plasma cells with positive staining for CD138 and lambda-
immunohistochemistry in the majority of plasma cells (Fig. 3).
Kappa-immunohistochemistry was negative in the majority of
plasma cells. In situ hybridization was negative for Epstein–Barr
virus and HHV8 in neoplastic cells. There was strong diffuse IgD
staining of the neoplastic cells, which correlated with the patient’s
known plasma cell multiple myeloma IgD variant.
FIGURE 2. MRI demonstration of the left medial canthal
episcleral soft tissue mass.
250 | www.corneajrnl.com
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Cornea  Volume 36, Number 2, February 2017
DISCUSSION
Multiple myeloma is malignant proliferation of plasma
cells in the bone marrow. Plasma cells are the most mature
form of B lymphocytes and are responsible for encoding the
arrangement of mature immunoglobulins. In multiple mye-
loma, there is abnormal propagation of monoclonal immuno-
globulins and/or light chains in the bone marrow, which
results in the diagnostic finding of monoclonal hypergamma-
globulinemia. The International Myeloma Working Group
criteria require at least 10% plasma cells in the bone marrow
or biopsy of a plasmacytoma and the presence of end organ
damage for the diagnosis of multiple myeloma.2
Ocular manifestations associated with multiple mye-
loma are separated into 2 groups; those secondary to
hematologic abnormalities associated with plasma cell
dyscrasia and those that are the direct result of tumor
growth in ocular or surrounding structures. Because of this
pathophysiology, ophthalmic involvement may occur in
virtually any ocular structure. Review of the literature
describes tumor affecting the orbit, conjunctiva, uvea,
retina, lacrimal sac, and ciliary body.3–9 However, the
occurrence of an ocular plasmacytoma is rare and those
with conjunctival involvement are challenging to diag-
nose. Reported cases describe the appearance of lesions
with variable gross examinations including diffuse con-
junctival thickening and those which present as a well-
defined mass.7 Our patient presented with subconjunctival
hemorrhage likely obscuring the underlying growth.
Because of the inconsistent appearance of lesions, exam-
ination skills will often not suffice for diagnosis. To
differentiate a true plasmacytoma from an inflammatory
reaction, biopsy with histopathologic examination is
required for definitive diagnosis.
This case emphasizes the importance of maintaining
a high index of suspicion in patients with subconjunctival
hemorrhage with known or suspected multiple myeloma,
especially if the hemorrhage is recurrent. This patient with
known multiple myeloma presented with new-onset subcon-
junctival hemorrhage 4 months before observation of tumor
growth. We suspect that the subconjunctival hemorrhage
masked the presence of the plasmacytoma. The presence of
ophthalmic manifestations may be a sign of uncontrolled or
recurrent multiple myeloma. To our knowledge, there have
been no previous reports of subconjunctival hemorrhage
delaying detection of subconjunctival plasmacytoma as
described in our patient.
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Cornea  Volume 36, Number 2, February 2017
FIGURE 3. Immunohistochemical
stains of plasma cells: positive stain-
ing for CD138 (A) and lambda-
immunohistochemistry (B) in the
majority of plasma cells.
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