Annals of Hematology

, Volume 92, Issue 7, pp 989–992 | Cite as

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

  • Huai-Cheng Huang
  • Ann-Lii Cheng
  • Chung-Wu Lin
  • Sung-Hsin Kuo
Letter to the Editor

Dear Editor,

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 [1]. 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.

A 31-year-old man...


Malt Lymphoma Central Nervous System Lymphoma Central Nervous System Relapse Intraocular Lymphoma Central Nervous System Prophylaxis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Conflict of interest

No financial or other potential conflicts of interest exist for any of the authors.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Huai-Cheng Huang
    • 1
    • 3
  • Ann-Lii Cheng
    • 2
    • 3
    • 5
  • Chung-Wu Lin
    • 4
  • Sung-Hsin Kuo
    • 2
    • 3
    • 5
  1. 1.Department of Oncology, Yun-Lin BranchNational Taiwan University HospitalYun-LinTaiwan
  2. 2.Department of Internal MedicineNational Taiwan University Hospital and National Taiwan University College of MedicineTaipeiTaiwan
  3. 3.Department of OncologyNational Taiwan University HospitalTaipeiTaiwan
  4. 4.Department of PathologyNational Taiwan University Hospital and National Taiwan University College of MedicineTaipeiTaiwan
  5. 5.Cancer Research CenterNational Taiwan University Hospital and National Taiwan University College of MedicineTaipeiTaiwan

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