Skip Navigation LinksHome > Summer 2014 - Volume 8 - Issue 3 > POSTERIOR SEGMENT INVOLVEMENT IN CASTLEMAN DISEASE
RETINAL Cases & Brief Reports:
doi: 10.1097/ICB.0000000000000044
Case Report


Katz, Matthew S. J. MD*; Park, Sunju MD*; Gritz, David C. MD, MPH*,†

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Author Information

Departments of *Ophthalmology and Visual Science, and

Epidemiology and Population Health, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York.

Reprint requests: David C. Gritz, MD, MPH, Department of Ophthalmology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467; e-mail:

Supported in part by an unrestricted institutional grant award from The Research to Prevent Blindness Foundation.

Paper presented at Association for Research in Vision and Ophthalmology in Seattle, WA, May 9, 2013.

None of the authors have any conflicting interests to disclose.

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We present here a patient with Castleman disease, a rare lymphoproliferative disorder, involving the vitreous. Her clinical findings are described.


Retrospective case study with slit-lamp photographs. A 28-year-old woman with known Castleman disease presented with visual complaints. Her ophthalmic findings are described.

Results and Conclusion:

Based on literature searches, this is the first reported patient with Castleman disease having putative involvement of the vitreous body.

Castleman disease (CD) is a rare lymphoproliferative disorder that can be discrete or disseminated, occult or life-threatening. Aberrant proliferation of lymph tissue occurs most commonly in the lymph nodes, but only rarely at extranodal sites; rarer still is the proliferation at orbital or ocular structures. Based on literature searches, this is the first reported patient with CD having putative involvement of the vitreous body.

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Case Report

A 28-year-old Caribbean-born black woman with known multicentric plasma cell variant Castleman disease presented to the emergency department complaining of longstanding recurrent frontal headaches with acute exacerbation in the last 3 days. She had previously been treated with rituximab and oral corticosteroids and her CD was thought to be in remission. Her medical history was also remarkable for recurrent interstitial lung disease and deep venous thromboses and pulmonary emboli necessitating systemic anticoagulation. Blood drawn in the emergency room found a microcytic anemia (mean corpuscular volume, 66.5 fL; hematocrit, 31%), neutropenia (2.8 k/uL), and elevated inflammatory markers (erythrocyte sedimentation rate, 52 mm/hour), and she was admitted for evaluation.

The patient also reported insidious, painless decreased vision bilaterally. Examination found best-corrected visual acuity of 20/20 in both eyes and normal anterior segments. Dilation revealed anterior vitreous cell in both eyes (Figure 1) and snowballs in the periphery left eye (Figure 2). Her posterior segments were otherwise within normal limits.

Fig. 1
Fig. 1
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Fig. 2
Fig. 2
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After neuroimaging revealed no pathology to explain the headaches, they were attributed to and successfully treated as migraine. The patient's leukocytosis was ascribed to her recent use of trimethoprim–sulfamethoxazole in treatment for a cutaneous abscess; by the time of discharge, her leukocyte count had normalized. The patient's visual disturbance was attributed to her latent myopia, and was given corrective lenses.

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Castleman disease, or angiofollicular lymph node hyperplasia, is an uncommon and poorly understood lymphoproliferative disorder first described in 1954.1 The unicentric variant CD, often a slow-growing asymptomatic mass, was described first. Multicentric Castleman disease is characterized by wide distribution, systemic symptoms, and poor prognosis. Although resection of unicentric variant CD is curative, management of multicentric Castleman disease merits systemic therapy; systemic corticosteroids, chemotherapeutic agents, radiation, and immunomodulatory therapy have all been used with varying degrees of success.2

Histopathologically, CD is designated as either hyaline vascular variant, more common and characterized by the involvement of germinal centers, or plasma cell variant, rarer and characterized by sparing of germinal centers, or mixed cell type. Patients with CD frequently present with cytopenias and have been found to have elevated serum interleukin-6 concentrations.

Castleman disease only rarely involves orbital and ocular structures. This is not surprising, given the paucity of lymph tissue in the orbit. Orbital or ocular involvement has been reported in both unicentric and multicentric variants.

Patients with CD involving the orbit or the eye infrequently present with ocular symptoms. Patients may complain of diplopia, ptosis, lid swelling, or exophthalmos, only four previously reported cases however reported a decrease in vision.3–6

Orbital involvement constitutes an uncommon presentation of CD; infiltration of eyelids, lacrimal gland, and the extraocular muscles have been observed.3 Intraocular involvement however is far rarer; previously described cases report infiltration of the optic disk, chorioretinal lesions, lesions of the retinal pigment epithelium, exudative retinal detachment, retinal venous occlusion, anterior uveitis, and uveal effusion syndrome (Table 1).4–6 The patient reported in this case is the first patient with Castleman disease reported to have involvement of the vitreous body.

Table 1
Table 1
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1. Castleman B, Thowne VW. Case records of the Massachusetts General Hospital Weekly Clinicopathological Exercises: case 40011. N Engl J Med. 1954; 250:26–30.

2. Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005; 129:3–17.

3. Venizelos I, Papathomas TG, Papathanasiou M, et al. Orbital involvement in Castleman disease. Surv Ophthalmol. 2010; 55:247–255.

4. Gittinger JW Jr. Ocular involvement in Castleman's disease. Response to radiotherapy. Ophthalmology. 1989; 96:1646–1649.

5. Kim U, Hwang JM. Optic neuropathy associated with Castleman disease. Korean J Ophthalmol. 2010; 24:256–259.

6. Jorge R, Scott IU, Oliveira RC, et al. Ocular findings in a patient with Castleman's disease before and after treatment with immunosuppression and plasmapheresis. Ophthalmic Surg Lasers Imaging. 2010; 41:.

7. Oshitari T, Kajita F, Tobe A, et al. Refractory uveitis in patient with Castleman disease successfully treated with tocilizumab. Case Rep Ophthalmol Med. 2012; 2012:968180

8. Park SH, Song SJ. Castleman's disease presenting with uveal effusion syndrome. Korean J Ophthalmol. 2010; 24:182–185.

9. Liu DT, Shields JA, Li CL, et al. Hypertensive choroidopathy in Castleman's disease. Graefes Arch Clin Exp Ophthalmol. 2011; 249:1901–1903.

10. Kozak I, Reid EG. Retinal vein occlusion during flare of multicentric Castleman's disease. Clin Ophthalmol. 2013; 7:1647–1649.


uveitis; vitritis


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