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Clinicopathologic Reports, Case Reports, and Small Case Series |

Lymphomatoid Granulomatosis Associated With Bilateral Exudative Retinal Detachments FREE

James R. Cameron, MRCOphth; Peter Cackett, FRCOphth
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Section Editor: W. Richard Green, MD

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Arch Ophthalmol. 2007;125(5):712-713. doi:10.1001/archopht.125.5.712.
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Lymphomatoid granulomatosis (LYG) was first described by Liebow et al1 in 1972. It is a necrotizing lymphoproliferative disorder primarily affecting the lungs. The skin, kidneys, and nervous system may be involved, but ocular involvement is unusual. We present the case of a 39-year-old man who developed bilateral exudative macula detachments 16 years following onset of LYG. Prompt management with systemic steroids resulted in complete resolution with no long-term sequelae.

REPORT OF A CASE

A 39-year-old man attended the ophthalmology department with a 3-day history of bilateral blurring of vision with no other ocular symptoms. There was no ophthalmological history.

He had been examined because of cough, dyspnea, and a skin rash 16 years previously and was subsequently diagnosed with LYG. Chest x-ray film had shown bilateral infiltrates with bronchoscopic biopsy yielding granulomatous material with perivascular lymphoid infiltrates. A skin biopsy helped confirm the diagnosis of LYG. At the time of his initial ophthalmological examination, he was taking 20 mg of prednisolone daily to control the pulmonary symptoms of LYG.

On examination, his visual acuities were 20/60 OD and 20/40 OS. Fundus examination revealed bilateral dome-shaped smooth elevations of the macula with subretinal fluid visible, consistent with exudative retinal detachments. No retinal breaks, vasculitis, or vitritis was present. Fluorescein angiography demonstrated bilateral multifocal pinpoint leakage throughout the macula and extensive subretinal fluid (Figure).

Place holder to copy figure label and caption
Figure.

Late-stage fundal fluorescein angiogram of the patient's fundi revealing extensive bilateral macula leakage consistent with subretinal fluid.

Graphic Jump Location

The patient's dose of prednisolone was increased to 60 mg daily. Within 4 weeks, visual acuities had returned to 20/20 OU with normal Amsler test results. Fundus examination now revealed complete resolution of the subretinal fluid and flat retinae bilaterally with no obvious sequelae of the exudative detachments seen.

The dose of prednisolone was thereafter reduced in 5 mg increments, and 5 months later, with the dose back at 20 mg, vision had remained stable with no recurrence of the subretinal fluid. Two years of follow-up have seen no further ocular involvement, but further skin lesions and arthralgia in the patient have also responded to systemic steroid therapy.

COMMENT

Lymphomatoid granulomatosis is an uncommon granulomatous inflammatory syndrome closely related to Wegener granulomatosis and to malignant lymphoma. It requires histopathological diagnosis because its clinical features are often difficult to distinguish from those of other granulomatoses, so it is frequently misdiagnosed as sarcoidosis.2 Clinical features are usually fever, cough, malaise, weight loss, and dyspnea. Men are more affected than women (3:2), and it may appear at any age.

Systemic involvement is predominantly pulmonary (>80%) with both interstitial and alveolar tissue affected. Central and peripheral nervous system involvement is common with skin and renal tissue involvement also seen. Radiographically, multiple variable-sized nodules are seen within the lung fields. Histologically, these appear as regions of necrosis and granulomatous inflammation with focal areas of vascular infiltration by atypical lymphocytes (possibly malignant B cells) and plasma cells. This appearance has led to its dual classification as a primary vasculitis and also as a lymphoma precursor. The absence of multinucleated giant cells or palisading epithelioid cells excludes the diagnosis of Wegener granulomatosis. Etiology remains unclear; however, in situ hybridization techniques have confirmed the presence of Epstein-Barr virus in many pathological samples.3 Epstein-Barr virus is a known factor in the pathogenesis of nasopharyngeal carcinoma and a number of proliferative lymphoid conditions. Progression to lymphoma frequently occurs, carrying a very poor prognosis and an associated mortality of 60% to 90%.

Ocular involvement in LYG is rare but appears to follow either cranial nerve involvement (tonic pupil,4 optic neuropathy5) or direct ocular granulomatous inflammation (posterior uveitis,6 choroidal vasculitis7,8). Choroidal involvement of LYG has been described only as a vasculitis without breach of the blood-retinal barrier. Our case is unique in reporting the occurrence of subretinal fluid secondary to presumed choroidal involvement of LYG.

No controlled trials of treatment exist. Immunosuppression and chemotherapy have both been used to manage ophthalmological manifestations as well as pulmonary disease. Recent studies have looked at other possible treatments with some success demonstrated with monoclonal antibody therapy such as rituximab.9

This case represents an unusual presentation of LYG with bilateral exudative retinal detachments that showed prompt resolution with systemic steroid therapy. To the best of our knowledge, this is the first reported case of LYG manifesting in this way.

ARTICLE INFORMATION

Correspondence: Dr Cameron, Princess Alexandra Eye Pavilion, Chalmers Street, Edinburgh EH3 9HA, Scotland (docjim@gmail.com).

Financial Disclosure: None reported.

REFERENCES

Liebow  AACarrington  CBFriedman  PJ Lymphomatoid granulomatosis. Hum Pathol 1972;3457- 558
PubMed Link to Article
Fitch  PSSmith  MEFDavies  MGPrentice  AG A case of lymphomatoid granulomatosis mimicking sarcoidosis. Respir Med 1998;92966- 968
PubMed Link to Article
Loughrey  MTrivett  MLade  SMurray  WTurner  HWaring  P Diagnostic application of Epstein-Barr virus-encoded RNA in situ hybridisation. Pathology 2004;36301- 308
PubMed Link to Article
Haider  S Tonic pupil in lymphomatoid granulomatosis. J Clin Neuroophthalmol 1993;1338- 39
PubMed
Forman  SRosenbaum  PS Lymphomatoid granulomatosis presenting as an isolated unilateral optic neuropathy: a clinicopathologic report. J Neuroophthalmol 1998;18150- 152
PubMed Link to Article
Tse  DTMandelbaum  SChuck  DANichols  PWSmith  RE Lymphomatoid granulomatosis with ocular involvement. Retina 1985;594- 97
PubMed Link to Article
Pearson  ADCraft  AWHowe  JM Choroidal involvement in lymphomatoid granulomatosis. Br J Ophthalmol 1991;75688- 689
PubMed Link to Article
Kinyoun  JLKalina  REKlein  ML Choroidal involvement in systemic necrotizing vasculitis. Arch Ophthalmol 1987;105939- 942
PubMed Link to Article
Jordan  KGrothey  AGrothe  WKegel  TWolf  HHSchmoll  HJ Successful treatment of mediastinal lymphomatoid granulomatosis with rituximab monotherapy. Eur J Haematol 2005;74263- 266
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure.

Late-stage fundal fluorescein angiogram of the patient's fundi revealing extensive bilateral macula leakage consistent with subretinal fluid.

Graphic Jump Location

Tables

References

Liebow  AACarrington  CBFriedman  PJ Lymphomatoid granulomatosis. Hum Pathol 1972;3457- 558
PubMed Link to Article
Fitch  PSSmith  MEFDavies  MGPrentice  AG A case of lymphomatoid granulomatosis mimicking sarcoidosis. Respir Med 1998;92966- 968
PubMed Link to Article
Loughrey  MTrivett  MLade  SMurray  WTurner  HWaring  P Diagnostic application of Epstein-Barr virus-encoded RNA in situ hybridisation. Pathology 2004;36301- 308
PubMed Link to Article
Haider  S Tonic pupil in lymphomatoid granulomatosis. J Clin Neuroophthalmol 1993;1338- 39
PubMed
Forman  SRosenbaum  PS Lymphomatoid granulomatosis presenting as an isolated unilateral optic neuropathy: a clinicopathologic report. J Neuroophthalmol 1998;18150- 152
PubMed Link to Article
Tse  DTMandelbaum  SChuck  DANichols  PWSmith  RE Lymphomatoid granulomatosis with ocular involvement. Retina 1985;594- 97
PubMed Link to Article
Pearson  ADCraft  AWHowe  JM Choroidal involvement in lymphomatoid granulomatosis. Br J Ophthalmol 1991;75688- 689
PubMed Link to Article
Kinyoun  JLKalina  REKlein  ML Choroidal involvement in systemic necrotizing vasculitis. Arch Ophthalmol 1987;105939- 942
PubMed Link to Article
Jordan  KGrothey  AGrothe  WKegel  TWolf  HHSchmoll  HJ Successful treatment of mediastinal lymphomatoid granulomatosis with rituximab monotherapy. Eur J Haematol 2005;74263- 266
PubMed Link to Article

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