Journal of Experimental Hematology

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1 912 Journal of Experimental Hematology Article ID MALT * B MALT 8 MALT 3 ⅠE COP /CHOP 5 Ⅳ COP /CHOP CR PR 100% 3 ⅠE PR 5 Ⅳ CR 21 3 ⅠE PR 5 Ⅳ CR MALT MALT R R A Efficacy of Chemotherapy as a First-line Treatment in Patients with Ocular Adne xal MALT Lymphoma NING Feng YE Jin WEI Li-Qiang LI Xin WANG Jing-Wen * Department of Hematology Beijing Tongren Hospital Capital University of Medical Sciences Beijing China * Corresponding Author WANG Jing-Wen Senior Physician Tel trwangjw@ gmail. com Abstract The aim of this study was to analyze the efficacy of first-line chemotherapy in treating patients with ocular adnexal etranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue MALT. Eight consecutive newly diagnosed ocular adnexal MALT lymphoma patients were treated with chemotherapy in which 3 patients in stageⅠe were treated with a combination of cyclophosphamide vincristine and prednisolone COP or cyclophosphamide adriamycin vincristine and prednisolone CHOP 5 patients in stage Ⅳ were treated with COP / CHOP in combination with rituximab. The results showed that chemotherapy resulted in an overall response rate of 100% 3 patients in stageⅠe were in partial remission PR 5 patients in stage Ⅳ were in complete remission CR. After a median follow-up of 21 months 3 patients in stageⅠe were still in PR status 5 patients in stage Ⅳ were still in CR status and no relapses or disease progression were observed. It is concluded that the first-line chemotherapy has been confirmed to be effective and well tolerated in patients with localized ocular adnexal MALT lymphoma. Rituximab combined with chemotherapy can increase remission rate it is feasible option as first-line treatment for ocular adnexal MALT lymphoma. Key words MALT lymphoma ocular adnexal lymphoma chemotherapy J Exp Hematol ocular adnexal lymphoma 8 MALT OAL 55% NHL 2% 8% B extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue MALT 35% - 80% 1 2 MALT MALT 1 * trwangjw@ gmail. com

2 MALT 913 MALT WHO Table 1. Characteristics of 8 patients with ocular adnexal IPI etranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue type ESR LDH β2 β2-mg Characteristics Number of patients FCM MRI Sex 7 PET /CT Male 4 PET /CT CT Ann Arbor Female 4 COP 1000 mg /m mg /m 2 2 mg 100 mg CHOP 50 mg /m 2 R 375 mg /m CT MRI Systemic involvement PET /CT LDH β2-mg MRI Note median age year was history of orbital involvement WHO month was CR PR PD 1 Ⅳ SPSS Kaplan-Meier LDH β2-mg 2 Ⅳ ki-67 10% 2% - 18% 1 1 Ann Arbor stage ⅠE 3 Ⅳ 5 Orbital involvement Eyelid 2 Orbit + Eyelid 4 Orbit + Eyelid + Lachrymal gland 2 Bilatera 2 Nodes 2 Soft tissue 2 Bone marrow 2 B symptoms 0 Elevated lactate dehydrogenase 0 Elevated β2-microglobulin 2 100% 3 ⅠE 2 4 CHOP MRI PR Ⅰ E 3 Ⅳ 5 CHOP 3 PR 1 CHOP 4 ⅠE 3 PR FMD Ⅳmg /m mg /m mg /d 1-3 2

914 J Exp Hematol 2012 20 4 PR 5 R + CHOP 6 PET-CT Ⅳ 4 R + CHOP 8 1 79 CR 2 1 2 Table 2.

month 1 Male /48 ⅠE CHOP 4 PR 6 FMD 2 PR 2 Male /76 ⅠE COP 4 PR 12 3 Male /63 Ⅳ R-CHOP 8 CR 18 4 Male /41 ⅠE CHOP 7 PR 18 5 Female /79 Ⅳ R-COP 8 CR 24 6 Female /71 Ⅳ

3 914 J Exp Hematol PR 5 R + CHOP 6 PET-CT Ⅳ 4 R + CHOP CR Table 2. Treatment outcomes of 8 ocular adnexal MALT lymphoma patients treated with chemotherapy No Sex /age Stage First-line treatment Cycle of treatment Response Follow-up month 1 Male /48 ⅠE CHOP 4 PR 6 FMD 2 PR 2 Male /76 ⅠE COP 4 PR 12 3 Male /63 Ⅳ R-CHOP 8 CR 18 4 Male /41 ⅠE CHOP 7 PR 18 5 Female /79 Ⅳ R-COP 8 CR 24 6 Female /71 Ⅳ R-CHOP 8 CR 24 7 Female /62 Ⅳ R-CHOP 8 CR 35 8 Female /53 Ⅳ R-CHOP 8 CR 36 PR partial response CR complete response. Figure 1. PET / CT results of a patient before and after chemotherapy. Figure 2. MRT results of a patient before and after chemotherapy.

4 MALT 915 COP /CHOP COP /CHOP + R MALT MALT Ⅰ - Ⅱ 2 Ⅰ 5% - 10% FCM B PET /CT MALT FCM MALT ⅠE PR 5 Ⅳ MALT CR COP /CHOP + R NCCN CR P = MALT MALT MALT B 2001 WHO MALT MALT 12% 2 MALT % % 47% 1 /3 MALT % - 25% MALT 2 3 Ann Arbor NCCN MALT MALT Ⅰ E Gy 10% - 15% ⅠE MALT 30 Gy 4 ⅠE Ⅳ CR 85% - 100% 5 94% 7 PET /CT 10% - 25% 10 PET /CT 7 MALT 27% 50% 30% - 50% CT MRI PET /CT 86% vs 72% 71% 20% - 40% 36 Gy PET /CT 5 8 PET /CT PET /CT 3 Ⅰ PET /CT 3 Ⅰ

5 916 J Exp Hematol COP /CHOP 100% 3 CR 62% 6 100% 1 CR 90% CHOP 100% CR 76. 2% CHOP 1000 mg /m 2 40 mg / m COP /CHOP MALT COP /CHOP OAML COP /CHOP CR CR Ⅰ CR 12 MALT 1 Ferry JA Fung CY Zukerberg L et al. Lymphoma of the ocular adnexa a study of 353 cases. Am J Surg Pathol MALT Decaudin D de Cremoux P Vincent-Salomon A et al. Ocular adnexal lymphoma a review of clinicopathologic features and treatment options. Blood Thieblemont C Berger F Dumontet C et al. Mucosa-associated CD20 lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood Tsang RW Gospodarowicz MK Pintilie M et al. Stage I and II MALT MALT lymphoma results of treatment with radiotherapy. Int J Radiat Oncol Biol Phys MALT Ferreri 11 5 Sullivan TJ Valenzuela AA. Imaging features of ocular adnexal lymphoproliferative disease. Eye lond ORR 50% - 87% 6 Tanimoto K Kaneko A Suzuki S et al. Long-term follow-up results of no initial therapy for ocular adnexal MALT lymphoma. Ann Oncol CR % Sullivan 13 CHOP 4 MALT % 5 CR Ⅳ COP /CHOP CR 5 PET /CT CR 9 2 CR CHOP OAML 1 1 MALT 1 OAML 7 Fung CY Tarbell NJ Lucarelli MJ et al. Ocular adnexal lymphoma clinical behavior of distinct World Health Organization classification subtypes. Int J Radiat Oncol Biol Phys Radiat Oncol Biol Phys. 8 Stafford SL Kozelsky TF Garrity JA et al. Orbital lymphoma radiotherapy outcome and complications. Radiother Oncol Meunier J Lumbroso-Le Rouic L Vincent-Salomon A et al. Ophthalmologic and intraocular non-hodgkin's lymphoma a large single centre study of initial characteristics natural history and prognostic factors. Hematol Oncol Song EK Kim SY Kim TM et al. Efficacy of chemotherapy as a first-line treatment in ocular adnexal extranodal marginal zone B-cell lymphoma. Ann Oncol Ferreri AJ Ponzoni M Martinelli G et al. Rituximab in patients with mucosal-associated lymphoid tissue-type lymphoma of the ocular adnexa. Haematologica Rigacci L Nassi L Puccioni M et al. Rituximab and chlorambucil as first-line treatment for low-grade ocular adnexal lymphomas. Ann Hematol Sullivan TJ Grimes D Bunce Ian Monoclonal antibody treatment of orbital lymphoma. Ophthal Plast Reconstr Sur Salar A Domingo-Domenech E Estan C et al. Combination therapy with rituximab and intravenous or oral fludarabine in the first-line systemic treatment of patients with extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type. Cancer

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