J Rheum Dis.  2016 Feb;23(1):61-65. 10.4078/jrd.2016.23.1.61.

A Case of Non-Hodgkin's Lymphoma of the Ocular Adnexa and Lung in a 17-year-old with Primary Sjogren's Syndrome

• Kim SH
• Lee HA
• Park SW
• Lee KW
• Seo YH
• Kim JH
• Choi SJ
• Lee YH
• Ji JD
• Song GG

• KMID: 2222802
• DOI: http://doi.org/10.4078/jrd.2016.23.1.61

Abstract

Sjogren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocyte-mediated destruction of exocrine glands, which produces classical symptoms of dry eyes and dry mouth. Aside from the clinical manifestations associated with exocrine glands, extraglandular features of SS include a major long-term concern for development of lymphoma. The lifetime risk of non-Hodgkin's lymphoma (NHL) in an SS patient is approximately 5% to 10%, 20 times higher than that of the normal population. This case report describes a rare occurrence of NHL in the eyelid and lung of an adolescent female with SS, whose disease activity had been monitored closely. This is the first reported case in Korea.

Keyword

Sjogren's syndrome; Lymphoma; Conjunctiva; Lung

MeSH Terms

Adolescent*
Conjunctiva
Exocrine Glands
Eyelids
Female
Humans
Korea
Lung*
Lymphoma
Lymphoma, Non-Hodgkin*
Mouth
Sjogren's Syndrome*

Figure

• 

  Figure 1. Orbital computed tomography scan (A, B) showed no abnormal findings in the or-bits, eyeballs, extraocular muscles, or optic nerves.

• 

  Figure 2. Conjunctival lymphoid tissue. H&E stained sctions showing abnormal lymphoid architecture and atypical lymphoid cells with mitosis (A: ×40, B: ×400).

• 

  Figure 3. Immunohistochemistry showing extensive, dense, packed CD20 B-cell lymphoid infiltrate (immunohistochemistry with hematoxylin counterstain, ×400).

• 

  Figure 4. Multifocal patchy consolidations and ground glass opacities in the right middle lobe (arrow).

Reference

1. Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren's syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome. Arthritis Rheum. 1999; 42:1765–72.

2. Giannouli S, Voulgarelis M. Predicting progression to lymphoma in Sjögren's syndrome patients. Expert Rev Clin Immunol. 2014; 10:501–12.

3. Nocturne G, Mariette X. Sjögren syndrome-associated lymphomas: an update on pathogenesis and management. Br J Haematol. 2015; 168:317–27.

4. Hahn JS, Kim C, Min YH, Ko YW, Suh CO, Park YY. Non-Hodgkin's lymphoma & primary biliary cirrhosis with Sjögren's syndrome. Yonsei Med J. 2001; 42:258–63.

5. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. European Study Group on Classification Criteria for Sjögren's Syndrome. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002; 61:554–8.

6. Zelenetz AD, Gordon LI, Wierda WG, Abramson JS, Advani RH, Andreadis CB, et al. National comprehensive cancer network. Non-Hodgkin's lymphomas, version 4.2014. J Natl Compr Canc Netw. 2014; 12:1282–303.

7. Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjögren's syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006; 65:796–803.

8. Dong L, Chen Y, Masaki Y, Okazaki T, Umehara H. Possible mechanisms of lymphoma development in Sjögren's syndrome. Curr Immunol Rev. 2013; 9:13–22.

9. Sretenovic M, Colovic M, Jankovic G, Suvajdzic N, Mihaljevic B, Colovic N, et al. More than a third of non-gas-tric malt lymphomas are disseminated at diagnosis: a single center survey. Eur J Haematol. 2009; 82:373–80.

10. Thieblemont C, Berger F, Dumontet C, Moullet I, Bouafia F, Felman P, et al. Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood. 2000; 95:802–6.

11. McKelvie PA, McNab A, Francis IC, Fox R, O'Day J. Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Experiment Ophthalmol. 2001; 29:387–93.

12. Decaudin D, de Cremoux P, Vincent-Salomon A, Dendale R, Rouic LL. Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options. Blood. 2006; 108:1451–60.

13. Papiris SA, Kalomenidis I, Malagari K, Kapotsis GE, Harhalakis N, Manali ED, et al. Extranodal marginal zone B-cell lymphoma of the lung in Sjögren's syndrome patients: reappraisal of clinical, radiological, and pathology findings. Respir Med. 2007; 101:84–92.

14. Ahmed S, Kussick SJ, Siddiqui AK, Bhuiya TA, Khan A, Sarewitz S, et al. Bronchial-associated lymphoid tissue lymphoma: a clinical study of a rare disease. Eur J Cancer. 2004; 40:1320–6.

15. Kang HJ, Kim WS, Kim SJ, Lee JJ, Yang DH, Kim JS, et al. Phase II trial of rituximab plus CVP combination chemotherapy for advanced stage marginal zone lymphoma as a first-line therapy: Consortium for Improving Survival of Lymphoma (CISL) study. Ann Hematol. 2012; 91:543–51.