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首页> 外文期刊>British Journal of Haematology >Primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (MALT): single institution experience in a large cohort of patients.
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Primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (MALT): single institution experience in a large cohort of patients.

机译：原发性眼附属物粘膜相关淋巴样组织淋巴瘤（MALT）：在一大批患者中有单一机构的经验。

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摘要

Extranodal marginal zone B-cell lymphoma is the most common orbital tumour. We conducted a retrospective analysis to examine: (i) the impact of initial presentation and staging on outcome and (ii) response to various treatment modalities and the effect of the latter on recurrence. Ninety patients with primary ocular adnexal marginal zone lymphoma (POAML) diagnosed at our institution between 1984 and 2009 were studied. POAML was associated with monoclonal gammopathy (13%) at presentation. Most POAML patients (86%) presented with Ann-Arbor stage I disease. Radiotherapy led to excellent local control, but relapses occurred in 18% of Ann-Arbor stage I patients during a median follow-up of 5 years. Local relapses, including secondary central nervous system (CNS) involvement, were observed in patients receiving radiation doses <30·6 Gy. No differences in relapse rate and survival were observed between patients who did or did not undergo staging bone marrow biopsy. Ann-Arbor stage II-IV disease and high lactate dehydrogenase levels were associated with shorter freedom from progression. In conclusion, POAML is an indolent lymphoma with continuous risk for relapse. Radiation doses of at least 30·6 Gy should be given in Ann-Arbor stage I disease, since lower doses may be more frequently associated with relapses, including CNS relapses.
机译：结外边缘区B细胞淋巴瘤是最常见的眼眶肿瘤。我们进行了回顾性分析，以检查：（i）初次就诊和分期对结局的影响，以及（ii）对各种治疗方式的反应以及后者对复发的影响。我们研究了1984年至2009年间在我们机构诊断出的90例原发性眼附属物边缘区淋巴瘤（POAML）患者。介绍时POAML与单克隆性丙种球蛋白病（13％）相关。大多数POAML患者（86％）患有Ann-Arbor I期疾病。放疗可实现出色的局部控制，但在5年的中位随访期间，Ann-Arbor I期患者中有18％复发。接受辐射剂量<30·6 Gy的患者观察到局部复发，包括次级中枢神经系统（CNS）受累。在进行或未进行分期骨髓活检的患者之间，未观察到复发率和生存率的差异。 Ann-Arbor II-IV期疾病和高乳酸脱氢酶水平与疾病进展的自由度短有关。总之，POAML是一种惰性淋巴瘤，具有持续复发的风险。在Ann-Arbor I期疾病中，应给予至少30·6 Gy的放射剂量，因为较低的剂量可能与复发（包括中枢神经系统复发）相关。

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