Abstract
Extra nodal marginal zone B-cell lymphoma (EMZL) of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue. The pathogenesis of conjunctival EMZL remains unknown. We describe an unusual case of EMZL arising from reactive lymphoid hyperplasia (RLH) of the conjunctiva. A 35-year-old woman had fleshy salmon-pink conjunctival tumors in both eyes, oculus uterque (OU). Specimens from conjunctival tumors in the right eye, oculus dexter (OD), revealed a collection of small lymphoid cells in the stroma. Immunohistochemically, immunoglobulin (Ig) light chain restriction was not detected. In contrast, diffuse atypical lymphoid cell infiltration was noted in the left eye, oculus sinister (OS), and positive for CD20, a marker for B cells OS. The tumors were histologically diagnosed as RLH OD, and EMZL OS. PCR analysis detected IgH gene rearrangement in the joining region (JH) region OU. After 11 months, a re-biopsy specimen demonstrated EMZL based on compatible pathological and genetic findings OD, arising from RLH. This case suggests that even if the diagnosis of the conjunctival lymphoproliferative lesions is histologically benign, confirmation of the B-cell clonality by checking IgH gene rearrangement should be useful to predict the incidence of malignancy.
Highlights
Extra nodal marginal zone B-cell lymphoma (EMZL) of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue
Previous studies reported that reactive lymphoid hyperplasia (RLH) of the orbit and eyelid developed systemic lymphomas in the selected cases [2,3]; there was no report showing EMZL of the conjunctiva which arose from RLH in situ
We recently reported a bilateral conjunctival lymphoproliferative disorder presenting with diffuse lymphoid cell infiltration with Ig heavy chain (IgH) gene rearrangement in one eye and without it in the contralateral eye [5]
Summary
Extra nodal marginal zone B-cell lymphoma (EMZL) of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue. Case presentation A 35-year-old woman had suffered from minor irritation in both eyes, oculus uterque (OU), and was treated with topical eye drops based on a diagnosis of allergic conjunctivitis at an initial clinic Since her conjunctival lesions did not improve, she was referred to our hospital on 17 December 2010. After the extraction of DNA from the fleshy unfixed conjunctival tumor tissues, PCR analysis detected Ig heavy chain (IgH) gene rearrangement in VH(FR1)/JH region OD and VH(FR2)/JH region OS. A histological examination showed diffuse atypical lymphoid cell infiltration, admixed with plasma cells OD (Figure 2C). The atypical lymphoid cells were positive for CD20 with deviation to kappa chains of Ig in the infiltrating lymphoid cells. Small lymphoid cells infiltrated the stroma OS (Figure 2D), where IgH gene rearrangement was not detected.
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