Primary central nervous system diffuse large B cell lymphoma transformed from orbital mucosa-associated lymphoid tissue lymphoma: complete response to combined intrathecal and systemic rituximab
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Extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT type) of the orbits accounts for 35–80 % of primary ocular adnexal lymphoma cases. The majority (85–90 %) of these patients present with localized disease and indolent behavior . Although localized ocular adnexal MALT lymphoma is highly responsive to radiotherapy, immunotherapy, and chemotherapy, a local relapse with high-grade transformation (most histology, diffuse large B cell lymphoma (DLBCL)) was rarely found in this subgroup of patients, with a reported rate of 2–3 % [2, 3]. In addition, primary ocular MALT lymphoma with central nervous system (CNS) involvement or relapse is rare, with most relapses developing locally or with distant recurrence [1, 4, 5]. This paper presents a localized MALT lymphoma of the right ocular adnexa that achieved complete remission after immunochemotherapy and early development of relapsed transformed high-grade DLBCL of the CNS.
KeywordsMalt Lymphoma Central Nervous System Lymphoma Central Nervous System Relapse Intraocular Lymphoma Central Nervous System Prophylaxis
Conflict of interest
No financial or other potential conflicts of interest exist for any of the authors.