Skip to main content

Advertisement

Log in

Primary intraocular lymphoma of T-cell type: report of a case and review of the literature

  • Review Article
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

Primary intraocular lymphoma (PIOL) is an uncommon non-Hodgkin lymphoma and is usually of B-cell type. Intraocular T-cell or T/NK-cell lymphomas are extremely rare and mostly represent a secondary manifestation of either a cutaneous or a systemic lymphoma. The aim of the current paper is to report the clinical, histopathological and molecular biological findings of a PIOL of T-cell type.

Methods

Conventional cytological and immunocytological examination of vitrectomy specimens. Conventional histology, immunohistochemistry and polymerase chain reaction (PCR) for the detection of immunoglobulin heavy chain (IgH) and T-cell-receptor gamma (TCR-γ) gene rearrangement, GeneScan analysis, and DNA sequencing were performed on the chorioretinal biopsy.

Results

Cytology of the right vitreous aspirate revealed a moderate cellular infiltrate consisting of medium-sized T-cells with pleomorphic nuclei. Similar atypical lymphocytes were seen in the partially necrotic chorioretinal biopsy. These lymphocytes expressed CD3, CD4, βF1 and CD30, with a growth fraction of 90%. TCR-γ-PCR, GeneScan analysis and DNA sequencing demonstrated a monoclonal amplification product within the expected range. In contrast, IgH-PCR revealed oligoclonal amplificates. The patient was treated with low-dose radiotherapy (total 45 Gy), and was in complete remission at final follow-up.

Conclusion

A rare PIOL of T-cell type was diagnosed on the basis of vitreous aspiration and chorioretinal biopsy. In addition to conventional cytology and immunocytology, the utilisation of gene rearrangement studies on vitreous or chorioretinal biopsies increases the chances of diagnosing or excluding a PIOL of either B-cell or T-cell type. Despite its rarity, ophthalmic pathologists should always consider the diagnosis of T-PIOL when reviewing vitreous samples.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Abrey LE, DeAngelis LM, Yahalom J (1998) Long-term survival in primary CNS lymphoma. J Clin Oncol 16:859–863

    CAS  PubMed  Google Scholar 

  2. Appen RE (1975) Posterior uveitis and primary cerebral reticulum cell sarcoma. Arch Ophthalmol 93:123–124

    CAS  PubMed  Google Scholar 

  3. Barr CC, Green WR, Payne JW et al (1975) Intraocular reticulum-cell sarcoma: clinico-pathologic study of four cases and review of the literature. Surv Ophthalmol 19:224–239

    CAS  PubMed  Google Scholar 

  4. Ben-Ezra D, Sahel JA, Harris NL et al (1989) Uveal lymphoid infiltrates: immunohistochemical evidence for a lymphoid neoplasia. Br J Ophthalmol 73:846–851

    CAS  PubMed  Google Scholar 

  5. Blumenkranz MS, Ward T, Murphy S et al (1992) Applications and limitations of vitreoretinal biopsy techniques in intraocular large cell lymphoma. Retina 12:S64–S70

    CAS  PubMed  Google Scholar 

  6. Brown SM, Jampol LM, Cantrill HL (1994) Intraocular lymphoma presenting as retinal vasculitis. Surv Ophthalmol 39:133–140

    Article  CAS  PubMed  Google Scholar 

  7. Cassoux N, Merle-Beral H, Leblond V et al (2000) Ocular and central nervous system lymphoma: clinical features and diagnosis. Ocul Immunol Inflamm 8:243–250

    Article  CAS  PubMed  Google Scholar 

  8. Cassoux N, Charlotte F, Rao NA et al (2004) Endoretinal biopsy in establishing the diagnosis of uveitis entities: clinicopathological report of three cases. Ocul Immunol Inflamm (in press)

    Google Scholar 

  9. Chan CC, Whitcup SM, Solomon D, Nussenblatt RB (1995) Interleukin-10 in the vitreous of patients with primary intraocular lymphoma. Am J Ophthalmol 120:671–673

    CAS  PubMed  Google Scholar 

  10. Char DH, Ljung BM, Deschenes J, Miller TR (1988) Intraocular lymphoma: immunological and cytological analysis. Br J Ophthalmol 72:905–911

    CAS  PubMed  Google Scholar 

  11. Char DH, Ljung BM, Miller T, Phillips T (1988) Primary intraocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management. Ophthalmology 95:625–630

    CAS  PubMed  Google Scholar 

  12. Ciulla TA, Bains RA, Jakobiec FA et al (1997) Uveal lymphoid neoplasia: a clinical-pathologic correlation and review of the early form. Surv Ophthalmol 41:467–476

    Article  CAS  PubMed  Google Scholar 

  13. Ciulla TA, Pesavento RD, Yoo S (1997) Subretinal aspiration biopsy of ocular lymphoma. Am J Ophthalmol 123:420–422

    CAS  PubMed  Google Scholar 

  14. Cochereau I, Hannouche D, Geoffray C et al (1996) Ocular involvement in Epstein-Barr virus-associated T-cell lymphoma. Am J Ophthalmol 121:322–324

    CAS  PubMed  Google Scholar 

  15. Cockerham GC, Hidayat AA, Bijwaard KE, Sheng ZM (2000) Re-evaluation of “reactive lymphoid hyperplasia of the uvea”: an immunohistochemical and molecular analysis of 10 cases. Ophthalmology 107:151–158

    Article  CAS  PubMed  Google Scholar 

  16. Cooper EL, Riker JL (1951) Malignant lymphoma of the uveal tract. Am J Ophthalmol 34:1153–1158

    CAS  PubMed  Google Scholar 

  17. Cordell JL, Falini B, Erber WN et al (1984) Immunoenzymatic labeling of monoclonal antibodies using immune complexes of alkaline phosphatase and monoclonal anti-alkaline phosphatase (APAAP complexes). J Histochem Cytochem 32:219–229

    CAS  PubMed  Google Scholar 

  18. Coupland SE, Foss HD, Assaf C et al (1999) T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases. Ophthalmology 106:2109–2120

    Article  CAS  PubMed  Google Scholar 

  19. Coupland SE, Foss HD, Bechrakis NE et al (2001) Secondary ocular involvement in systemic “memory” B-cell lymphocytic leukemia. Ophthalmology 108:1289–1295

    Article  CAS  PubMed  Google Scholar 

  20. Coupland SE, Foss HD, Hidayat AA et al (2002) Extranodal marginal zone B cell lymphomas of the uvea: an analysis of 13 cases. J Pathol 197:333–340

    Article  PubMed  Google Scholar 

  21. Coupland SE, Bechrakis NE, Anastassiou G et al (2003) Evaluation of vitrectomy specimens in the diagnosis of primary intraocular lymphoma. Graefes Arch Clin Exp Ophthalmol 241(10):860–870

    Article  PubMed  Google Scholar 

  22. Davis JL, Viciana AL, Ruiz P (1997) Diagnosis of intraocular lymphoma by flow cytometry. Am J Ophthalmol 124:362–372

    CAS  PubMed  Google Scholar 

  23. Erny BC, Egbert PR, Peat IM et al (1991) Intraocular involvement with subretinal pigment epithelium infiltrates by mycosis fungoides. Br J Ophthalmol 75:698–701 (published erratum appears in Br J Ophthalmol Feb;76(2):128)

    CAS  PubMed  Google Scholar 

  24. Fine HA, Mayer RJ (1993) Primary central nervous system lymphoma. Ann Intern Med 119:1093–1104

    CAS  PubMed  Google Scholar 

  25. Foerster H (1960) Mycosis fungoides with intraocular involvement. Trans Am Acad Ophthalmol Otolaryngol 64:308–314

    CAS  PubMed  Google Scholar 

  26. Freeman LN, Schachat AP, Knox DL et al (1987) Clinical features, laboratory investigations, and survival in ocular reticulum cell sarcoma. Ophthalmology 94:1631–1639

    CAS  PubMed  Google Scholar 

  27. Gärtner J (1989) Mycosis fungoides mit Beteiligung der Aderhaut. Klin Monatsbl Augenheilkd 131:61–69

    Google Scholar 

  28. Gass JD, Trattler HL (1991) Retinal artery obstruction and atheromas associated with non-Hodgkin’s large cell lymphoma (reticulum cell sarcoma). Arch Ophthalmol 109:1134–1139

    CAS  PubMed  Google Scholar 

  29. Gass JD, Sever RJ, Grizzard WS et al (1984) Multifocal pigment epithelial detachments by reticulum cell sarcoma. A characteristic funduscopic picture. Retina 4:135–143

    CAS  PubMed  Google Scholar 

  30. Gass JD, Weleber RG, Johnson DR (1987) Non-Hodgkin’s lymphoma causing fundus picture simulating fundus flavimaculatus. Retina 7:209–214

    CAS  PubMed  Google Scholar 

  31. Gill MK, Jampol LM (2001) Variations in the presentation of primary intraocular lymphoma: case reports and a review. Surv Ophthalmol 45:463–471

    Article  CAS  PubMed  Google Scholar 

  32. Goeminne JC, Brouillard A, Jaumain P et al (1999) Bilateral granulomatous panuveitis as initial presentation of diffuse systemic T cell lymphoma. Ophthalmologica 213:323–326

    Article  CAS  PubMed  Google Scholar 

  33. Goldey SH, Stern GA, Oblon DJ et al (1989) Immunophenotypic characterization of an unusual T-cell lymphoma presenting as anterior uveitis. A clinicopathologic case report. Arch Ophthalmol 107:1349–1353

    CAS  PubMed  Google Scholar 

  34. Grossniklaus HE, Martin DF, Avery R et al (1998) Uveal lymphoid infiltration. Report of four cases and clinicopathologic review. Ophthalmology 105:1265–1273

    Article  CAS  PubMed  Google Scholar 

  35. Hoffman PM, McKelvie P, Hall AJ et al (2003) Intraocular lymphoma: a series of 14 patients with clinicopathological features and treatment outcomes. Eye 17:513–521

    Article  CAS  PubMed  Google Scholar 

  36. Hsu SM, Raine L, Fanger H (1981) Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques: a comparison between ABC and unlabeled antibody (PAP) procedures. J Histochem Cytochem 29:577–580

    CAS  PubMed  Google Scholar 

  37. Jaffe ES, Harris NL, Stein H, Vardiman JW (2001) World Health Organization Classification of Tumours. Tumours of haematopoietic and lymphoid tissues. Pathology and genetics. IARC, Lyon

    Google Scholar 

  38. Jensen OA, Johansen S, Kiss K (1994) Intraocular T-cell lymphoma mimicking a ring melanoma. First manifestation of systemic disease. Report of a case and survey of the literature. Graefes Arch Clin Exp Ophthalmol 232:148–152

    Article  CAS  PubMed  Google Scholar 

  39. Kaplan HJ, Meredith TA, Aaberg TM, Keller RH (1980) Reclassification of intraocular reticulum cell sarcoma (histiocytic lymphoma). Immunologic characterization of vitreous cells. Arch Ophthalmol 98:707–710

    CAS  PubMed  Google Scholar 

  40. Katai N, Kuroiwa S, Fujimori K, Yoshimura N (1997) Diagnosis of intraocular lymphoma by polymerase chain reaction. Graefes Arch Clin Exp Ophthalmol 235:431–436

    CAS  PubMed  Google Scholar 

  41. Kawai Y, Kawai R, Nakayama T et al (1999) Successful treatment of cytomegalovirus retinitis in a patient with malignant lymphoma: a case report and review of the literature. Int J Hematol 69:256–259

    CAS  PubMed  Google Scholar 

  42. Keltner JL, Fritsch E, Cykiert RC, Albert DM (1977) Mycosis fungoides. Intraocular and central nervous system involvement. Arch Ophthalmol 95:645–650

    CAS  PubMed  Google Scholar 

  43. Kincaid ME, Green WR (1983) Ocular and orbital involvement in leukemia. Surv Ophthalmol 27:211–232

    Article  CAS  PubMed  Google Scholar 

  44. Kohno T, Uchida H, Inomata H et al (1993) Ocular manifestations of adult T-cell leukemia/lymphoma. A clinicopathologic study. Ophthalmology 100:1794–1799

    CAS  PubMed  Google Scholar 

  45. Kumar SR, Gill PS, Wagner DG et al (1994) Human T-cell lymphotropic virus type I-associated retinal lymphoma. A clinicopathologic report. Arch Ophthalmol 112:954–959

    CAS  PubMed  Google Scholar 

  46. Lafaut BA, Hanssens M, Verbraeken H, De Laey JJ (1994) Chorioretinal biopsy in the diagnosis of intraocular lymphoma: a case report. Bull Soc Belge Ophtalmol 252:67–73

    CAS  PubMed  Google Scholar 

  47. Leitch RJ, Rennie IG, Parsons MA (1993) Ocular involvement in mycosis fungoides. Br J Ophthalmol 77:126–127

    CAS  PubMed  Google Scholar 

  48. Lobo A, Larkin G, Clark BJ et al (2003) Pseudo-hypopyon as the presenting feature in B-cell and T-cell intraocular lymphoma. Clin Experiment Ophthalmol 31:155–158

    Article  PubMed  Google Scholar 

  49. Lois N, Hiscott PS, Nash J, Wong D (2000) Immunophenotypic shift in a case of mycosis fungoides with vitreous invasion. Arch Ophthalmol 118:1692–1694

    CAS  PubMed  Google Scholar 

  50. Martin DF, Chan CC, de Smet MD et al (1993) The role of chorioretinal biopsy in the management of posterior uveitis. Ophthalmology 100:705–714

    CAS  PubMed  Google Scholar 

  51. Merle-Beral H, Davi F, Cassoux N et al (2004) Biological diagnosis of primary intraocular lymphoma. Br J Haematol 124:469–473

    PubMed  Google Scholar 

  52. Norton AJ, Jordan S, Yeomans P (1994) Brief, high-temperature heat denaturation (pressure cooking): a simple and effective method of antigen retrieval for routinely processed tissues. J Pathol 173:371–379

    CAS  PubMed  Google Scholar 

  53. Pavan PR, Oteiza EE, Margo CE (1995) Ocular lymphoma diagnosed by internal subretinal pigment epithelium biopsy. Arch Ophthalmol 113:1233–1234

    CAS  PubMed  Google Scholar 

  54. Peterson K, Gordon KB, Heinemann MH, DeAngelis LM (1993) The clinical spectrum of ocular lymphoma. Cancer 72:843–849

    CAS  PubMed  Google Scholar 

  55. Qualman SJ, Mendelsohn G, Mann RB, Green WR (1983) Intraocular lymphomas. Natural history based on a clinicopathologic study of eight cases and review of the literature. Cancer 52:878–886

    CAS  PubMed  Google Scholar 

  56. Read RW, Zamir E, Rao NA (2002) Neoplastic masquerade syndromes. Surv Ophthalmol 47:81–124

    Article  PubMed  Google Scholar 

  57. Reim H, Dieler R, Wessing A (1990) Non-Hodgkin’s lymphoma simulating chorioretinitis. Fortschr Ophthalmol 87:557–559

    CAS  PubMed  Google Scholar 

  58. Ridley ME, McDonald HR, Sternberg P Jr et al (1992) Retinal manifestations of ocular lymphoma (reticulum cell sarcoma). Ophthalmology 99:1153–1160

    CAS  PubMed  Google Scholar 

  59. Rockwood EJ, Zakov ZN, Bay JW (1984) Combined malignant lymphoma of the eye and CNS (reticulum-cell sarcoma). Report of three cases. J Neurosurg 61:369–374

    CAS  PubMed  Google Scholar 

  60. Rothova A, Ooijman F, Kerkhoff F et al (2001) Uveitis masquerade syndromes. Ophthalmology 108:386–399

    Article  CAS  PubMed  Google Scholar 

  61. Saga T, Ohno S, Matsuda H et al (1984) Ocular involvement by a peripheral T-cell lymphoma. Arch Ophthalmol 102:399–402

    CAS  PubMed  Google Scholar 

  62. de Smet MD, Nussenblatt RB, Davis JL, Palestine AG (1990) Large cell lymphoma masquerading as a viral retinitis. Int Ophthalmol 14:413–417

    PubMed  Google Scholar 

  63. Soussain C, Suzan F, Hoang-Xuan K et al (2001) Results of intensive chemotherapy followed by hematopoietic stem-cell rescue in 22 patients with refractory or recurrent primary CNS lymphoma or intraocular lymphoma. J Clin Oncol 19:742–749

    CAS  PubMed  Google Scholar 

  64. Stenson S, Ramsay DL (1981) Ocular findings in mycosis fungoides. Arch Ophthalmol 99:272–277

    CAS  PubMed  Google Scholar 

  65. Wender A, Adar A, Maor E, Yassur Y (1994) Primary B-cell lymphoma of the eyes and brain in a 3-year-old boy. Arch Ophthalmol 112:450–451

    CAS  PubMed  Google Scholar 

  66. Whitcup SM, de Smet MD, Rubin BI et al (1993) Intraocular lymphoma. Clinical and histopathologic diagnosis. Ophthalmology 100:1399–1406

    CAS  PubMed  Google Scholar 

  67. Whitcup SM, Stark-Vancs V, Wittes RE et al (1997) Association of interleukin 10 in the vitreous and cerebrospinal fluid and primary central nervous system lymphoma. Arch Ophthalmol 115:1157–1160

    CAS  PubMed  Google Scholar 

  68. White VA, Gascoyne RD, Paton KE (1999) Use of the polymerase chain reaction to detect B- and T-cell gene rearrangements in vitreous specimens from patients with intraocular lymphoma. Arch Ophthalmol 117:761–765

    CAS  PubMed  Google Scholar 

  69. Williams GC, Holz E, Lee AG, Font RL (2000) T-cell lymphoproliferative disorder of vitreous associated with mycosis fungoides. Arch Ophthalmol 118:278–280

    CAS  PubMed  Google Scholar 

  70. Wolter J, Leenhouts T, Hendrix R (1963) Corneal involvement in mycosis fungoides. Am J Ophthalmol 55:315–322

    Google Scholar 

  71. Wylen EL, Williams RB, Nanda A (1998) Cutaneous T-cell lymphoma with intracerebral and bilateral intraocular spread. Neurol Res 20:307–312

    CAS  PubMed  Google Scholar 

  72. Yahalom C, Cohen Y, Averbukh E et al (2002) Bilateral iridociliary T-cell lymphoma. Arch Ophthalmol 120:204–207

    PubMed  Google Scholar 

  73. Young TL, Himelstein BP, Rebsamen SL et al (1996) Intraocular Ki-1 lymphoma in a 2-year-old boy. J Pediatr Ophthalmol Strabismus 33:268–270

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Sarah E. Coupland.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Coupland, S.E., Anastassiou, G., Bornfeld, N. et al. Primary intraocular lymphoma of T-cell type: report of a case and review of the literature. Graefe's Arch Clin Exp Ophthalmol 243, 189–197 (2005). https://doi.org/10.1007/s00417-004-0890-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00417-004-0890-2

Keywords

Navigation