MALT lymphoma of the lacrimal gland in the context of systemic lupus erythematosus: complete remission after treatment with rituximab

Abstract

Mucosa-associated lymphoid tissue (MALT) lymphoma of the lacrimal glands has been reported in only two patients with systemic lupus erythematosus (SLE) in the literature. We describe a 41-year-old female with SLE who had multiple relapses and remissions of her disease during the last 20 years and developed a right eyelid swelling. Magnetic resonance imaging showed a lesion in the right lacrimal gland with increased enhancement on T1- and T2-weighted images after intravenous contrast administration, and the biopsy of lacrimal gland was consistent with the diagnosis of marginal zone B-cell lymphoma of MALT type. The patient received treatment with four once-weekly doses of rituximab 375 mg/m² every 6 months for 2 years resulting in complete remission. Lacrimal gland MALT lymphoma is mainly treated with local radiotherapy, or chemotherapy in cases with systemic lymphoma. This is the first case of rituximab treatment in a patient with SLE who developed lacrimal gland MALT lymphoma, resulting in complete durable remission.

Keywords

lymphoma of lacrimal glands MALT lymphoma rituximab systemic lupus erythematosus

Get full access to this article

View all access options for this article.

References

Hainsworth JD , Litchy S. , Burris III HA , et al. Rituximab as first-line and maintenance therapy for patients with indolent non-Hodgkin’s lymphoma. J Clin Oncol 2002; 20: 4261-4267.

Ghielmini M. Multimodality therapies and optimal schedule of antibodies: rituximab in lymphoma as an example. Hematology Am Soc Hematol Educ Program 2005; 321-328.

Ramos-Casals M. , Soto MJ , Cuadrado MJ , Khamashta MA Rituximab in systemic lupus erythematosus: a systematic review of off-label use in 188 cases. Lupus 2009; 18: 767-776.

Lee JL , Kim MK , Lee KH , et al. Extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue-type of the orbit and ocular adnexa. Ann Hematol 2005; 84: 13-18.

Hansen A. , Lipsky PE , Dörner T. B-cell lymphoproliferation in chronic inflammatory rheumatic diseases. Nat Clin Pract Rheumatol 2007; 3: 561-569.

Voulgarelis M. , Skopouli FN Clinical, immunologic, and molecular factors predicting lymphoma development in Sjögren’s syndrome patients . Clin Rev Allergy Immunol 2007; 32: 265-274.

Martin DN , Mikhail IS , Landgren O. Autoimmunity and hematologic malignancies: associations and mechanisms. Leuk Lymphoma 2009; 50: 541-550.

Tonami H. , Matoba M. , Yokota H. , Higashi K. , Yamamoto I. , Sugai S. Mucosa-associated lymphoid tissue lymphoma in Sjögren’s syndrome: initial and follow-up imaging features. AJR Am J Roentgenol 2002; 179: 485-489.

Cassidy DT , McKelvie P. , Harris GJ , Rose GE , McNab AA Lacrimal gland orbital lobe cysts associated with MALT lymphoma and primary Sjögren’s syndrome. Orbit 2005; 24: 257-263.

10.

Kíratlí H. , Söylemezoğlu F. , Bilgiç S. , Ruacan S. Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland. Ophthal Plast Reconstr Surg 1999; 15: 272.

11.

Lin YC , Kau HC , Kao SC , Hsu WM , Tsai CC Systemically disseminated extranodal marginal zone B-cell lymphoma of lacrimal gland in a patient with systemic lupus erythematosus. Ophthal Plast Reconstr Surg 2007; 23: 72-73.

12.

Heinz C. , Merz H. , Nieschalk M. , Mueller-Miny H. , Koch P. , Heiligenhaus A. Rituximab for the treatment of extranodal marginal zone B-cell lymphoma of the lacrimal gland. Br J Ophthalmol 2007; 91: 1563-1564.

13.

Pijpe J. , van Imhoff GW , Spijkervet FK , et al. Rituximab treatment in patients with primary Sjögren’s syndrome: an open-label phase II study. Arthritis Rheum 2005; 52: 2740-2750.